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UniProtKB - P36776 (LONM_HUMAN)

Entry version 205 (02 Jun 2021)
Sequence version 2 (01 Dec 2000)
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Protein

Lon protease homolog, mitochondrial

Gene

LONP1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters (PubMed:12198491, PubMed:15870080, PubMed:17420247, PubMed:8248235).

Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575).

UniRule annotation6 Publications

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Peptidase activity is subject to substrate inhibition by ATP.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei855UniRule annotation1
Active sitei898UniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi523 – 530ATPUniRule annotation8

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

Complete GO annotation on QuickGO ...

GO - Biological processi

Complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionDNA-binding, Hydrolase, Protease, Serine protease
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

More...BRENDAi		3.4.21.53, 2681

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P36776

Protein family/group databases

MEROPS protease database

More...MEROPSi		S16.002

MoonDB Database of extreme multifunctional and moonlighting proteins

More...MoonDBi		P36776, Curated

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Lon protease homolog, mitochondrialUniRule annotation (EC:3.4.21.53UniRule annotation)
Alternative name(s):
LONHs
Lon protease-like proteinUniRule annotation
Short name:
LONPUniRule annotation
Mitochondrial ATP-dependent protease LonUniRule annotation
Serine protease 15UniRule annotation
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:LONP1UniRule annotation
Synonyms:PRSS15
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 19

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:9479, LONP1

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		605490, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P36776

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000196365.11

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

CODAS syndrome (CODASS)2 Publications
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionA rare syndrome characterized by the combination of cerebral, ocular, dental, auricular, and skeletal features. These include developmental delay, craniofacial anomalies, cataracts, ptosis, median nasal groove, delayed tooth eruption, hearing loss, short stature, delayed epiphyseal ossification, metaphyseal hip dysplasia, and vertebral coronal clefts.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_073338476E → A in CODASS. 1 Publication1
Natural variantiVAR_073339631S → Y in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255248EnsemblClinVar.1
Natural variantiVAR_073340670A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs770036526Ensembl.1
Natural variantiVAR_073341672R → C in CODASS. 1 PublicationCorresponds to variant dbSNP:rs777009012Ensembl.1
Natural variantiVAR_073342676P → S in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255247EnsemblClinVar.1
Natural variantiVAR_073343679R → H in CODASS. 1 PublicationCorresponds to variant dbSNP:rs549574673EnsemblClinVar.1
Natural variantiVAR_073344721R → G in CODASS. 1 PublicationCorresponds to variant dbSNP:rs147588238Ensembl.1
Natural variantiVAR_073345724A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255249EnsemblClinVar.1
Natural variantiVAR_073346749P → S in CODASS. 1 Publication1
Natural variantiVAR_073347767G → E in CODASS. 1 PublicationCorresponds to variant dbSNP:rs562553348Ensembl.1
Natural variantiVAR_073348927Missing in CODASS. 1 Publication1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi529K → R: Abolishes ATPase activity, and presumably ATP-driven protein unfolding, but does not block access to the proteolytic active site or prevent a substrate from binding to it. 1 Publication1
Mutagenesisi770W → A: Has low basal, but normal stimulated ATPase activity, and retains peptidase activity. 1 Publication1
Mutagenesisi770W → P: Has normal basal, but low stimulated ATPase activity, and abolishes peptidase activity. 1 Publication1
Mutagenesisi855S → A: Lacks both ATPase and protease activity, but retains DNA binding activity. 2 Publications1
Mutagenesisi880T → V: Enhances the basal, but not the stimulated ATPase activity. 1 Publication1
Mutagenesisi893G → A: Has low basal, but normal stimulated ATPase activity, and retains peptidase activity. 1 Publication1
Mutagenesisi893G → P: Has normal basal, but low stimulated ATPase activity, and abolishes peptidase activity. 1 Publication1
Mutagenesisi894G → A or S: Enhances the basal, but not the stimulated ATPase activity, and retains peptidase activity. 1 Publication1
Mutagenesisi894G → P: Enhances the basal, but not the stimulated ATPase activity, and abolishes peptidase activity. 1 Publication1

Keywords - Diseasei

Cataract, Deafness, Disease variant, Dwarfism

Organism-specific databases

DisGeNET

More...DisGeNETi		9361

MalaCards human disease database

More...MalaCardsi		LONP1
MIMi600373, phenotype

Open Targets

More...OpenTargetsi		ENSG00000196365

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		1458, CODAS syndrome
79243, Pyruvate dehydrogenase E1-alpha deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA162394145

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P36776, Tbio

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		LONP1

Domain mapping of disease mutations (DMDM)

More...DMDMi		12644239

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 67MitochondrionUniRule annotationCombined sourcesAdd BLAST67
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000002673468 – 959Lon protease homolog, mitochondrialAdd BLAST892

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P36776

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P36776

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P36776

MaxQB - The MaxQuant DataBase

More...MaxQBi		P36776

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P36776

PeptideAtlas

More...PeptideAtlasi		P36776

PRoteomics IDEntifications database

More...PRIDEi		P36776

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		24918
55221 [P36776-1]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P36776

MetOSite database of methionine sulfoxide sites

More...MetOSitei		P36776

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P36776

SwissPalm database of S-palmitoylation events

More...SwissPalmi		P36776

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the 'Expression' section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.<br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Duodenum, heart, lung and liver, but not thymus.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000196365, Expressed in right adrenal gland and 233 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P36776, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P36776, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000196365, Tissue enhanced (adrenal)

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homohexamer (PubMed:14739292, PubMed:25369343, PubMed:20222013). Organized in a ring with a central cavity (PubMed:25369343, PubMed:20222013). The ATP-binding and proteolytic domains (AP-domain) form a hexameric chamber, while the N-terminal domain is arranged as a trimer of dimers (PubMed:27632940). DNA and RNA binding is stimulated by substrate and inhibited by ATP binding.

Interacts with TWNK and mitochondrial DNA polymerase subunit POLG (PubMed:14739292).

UniRule annotation4 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Hide details

GO - Molecular functioni

Complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		114762, 293 interactors

Protein interaction database and analysis system

More...IntActi		P36776, 76 interactors

Molecular INTeraction database

More...MINTi		P36776

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000353826

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P36776, protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1959
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P36776

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		P36776

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family%5Fand%5Fdomains%5Fsection">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini124 – 368Lon N-terminalPROSITE-ProRule annotationAdd BLAST245
Domaini759 – 949Lon proteolyticPROSITE-ProRule annotationAdd BLAST191

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni77 – 102DisorderedSequence analysisAdd BLAST26
Regioni218 – 257DisorderedSequence analysisAdd BLAST40

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes the position of regions of compositional bias within the protein and the particular type of amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi218 – 233Basic and acidic residuesSequence analysisAdd BLAST16
Compositional biasi241 – 255Basic and acidic residuesSequence analysisAdd BLAST15

<p>This subsection of the 'Family and domains' section provides general information on the biological role of a domain. The term 'domain' is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

The Lon N-terminal domains are crucial for the overall structure of the protein, maintaining it in a conformation allowing its proper functioning.1 Publication
The AP-domain (ATP-binding and proteolytic domains) has a closed-ring conformation in the presence of AMP-PNP and its N-terminal entry gate appears closed. Upon ADP binding, it switches to a lock-washer conformation and its N-terminal gate opens.1 Publication
The proteolytic site is connected to the ATP binding site through the GG loop (Gly-893 and Gly-894) and the loop containing Trp-770. Binding of a protein substrate such as beta-casein appears to trigger movement of both these loops as part of the conformational changes which lead to enhanced ATPase and peptidase activities.1 Publication

<p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the peptidase S16 family.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG2004, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00530000063553

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		CLU_004109_1_1_1

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P36776

Identification of Orthologs from Complete Genome Data

More...OMAi		ENVAHED

Database of Orthologous Groups

More...OrthoDBi		528132at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P36776

TreeFam database of animal gene trees

More...TreeFami		TF105001

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.30.230.10, 1 hit

HAMAP database of protein families

More...HAMAPi		MF_03120, lonm_euk, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR003593, AAA+_ATPase
IPR003959, ATPase_AAA_core
IPR004815, Lon_bac/euk-typ
IPR008269, Lon_proteolytic
IPR027065, Lon_Prtase
IPR003111, Lon_substr-bd
IPR027503, Lonm_euk
IPR027417, P-loop_NTPase
IPR008268, Peptidase_S16_AS
IPR015947, PUA-like_sf
IPR020568, Ribosomal_S5_D2-typ_fold
IPR014721, Ribosomal_S5_D2-typ_fold_subgr

The PANTHER Classification System

More...PANTHERi		PTHR43718, PTHR43718, 1 hit

Pfam protein domain database

More...Pfami		View protein in Pfam
PF00004, AAA, 1 hit
PF05362, Lon_C, 1 hit
PF02190, LON_substr_bdg, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

More...SMARTi		View protein in SMART
SM00382, AAA, 1 hit
SM00464, LON, 1 hit

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF52540, SSF52540, 1 hit
SSF54211, SSF54211, 1 hit
SSF88697, SSF88697, 1 hit

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR00763, lon, 1 hit

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS51787, LON_N, 1 hit
PS51786, LON_PROTEOLYTIC, 1 hit
PS01046, LON_SER, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (3+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P36776-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MAASTGYVRL WGAARCWVLR RPMLAAAGGR VPTAAGAWLL RGQRTCDASP 
        60         70         80         90        100
PWALWGRGPA IGGQWRGFWE ASSRGGGAFS GGEDASEGGA EEGAGGAGGS 
       110        120        130        140        150
AGAGEGPVIT ALTPMTIPDV FPHLPLIAIT RNPVFPRFIK IIEVKNKKLV 
       160        170        180        190        200
ELLRRKVRLA QPYVGVFLKR DDSNESDVVE SLDEIYHTGT FAQIHEMQDL 
       210        220        230        240        250
GDKLRMIVMG HRRVHISRQL EVEPEEPEAE NKHKPRRKSK RGKKEAEDEL 
       260        270        280        290        300
SARHPAELAM EPTPELPAEV LMVEVENVVH EDFQVTEEVK ALTAEIVKTI 
       310        320        330        340        350
RDIIALNPLY RESVLQMMQA GQRVVDNPIY LSDMGAALTG AESHELQDVL 
       360        370        380        390        400
EETNIPKRLY KALSLLKKEF ELSKLQQRLG REVEEKIKQT HRKYLLQEQL 
       410        420        430        440        450
KIIKKELGLE KDDKDAIEEK FRERLKELVV PKHVMDVVDE ELSKLGLLDN 
       460        470        480        490        500
HSSEFNVTRN YLDWLTSIPW GKYSNENLDL ARAQAVLEED HYGMEDVKKR 
       510        520        530        540        550
ILEFIAVSQL RGSTQGKILC FYGPPGVGKT SIARSIARAL NREYFRFSVG 
       560        570        580        590        600
GMTDVAEIKG HRRTYVGAMP GKIIQCLKKT KTENPLILID EVDKIGRGYQ 
       610        620        630        640        650
GDPSSALLEL LDPEQNANFL DHYLDVPVDL SKVLFICTAN VTDTIPEPLR 
       660        670        680        690        700
DRMEMINVSG YVAQEKLAIA ERYLVPQARA LCGLDESKAK LSSDVLTLLI 
       710        720        730        740        750
KQYCRESGVR NLQKQVEKVL RKSAYKIVSG EAESVEVTPE NLQDFVGKPV 
       760        770        780        790        800
FTVERMYDVT PPGVVMGLAW TAMGGSTLFV ETSLRRPQDK DAKGDKDGSL 
       810        820        830        840        850
EVTGQLGEVM KESARIAYTF ARAFLMQHAP ANDYLVTSHI HLHVPEGATP 
       860        870        880        890        900
KDGPSAGCTI VTALLSLAMG RPVRQNLAMT GEVSLTGKIL PVGGIKEKTI 
       910        920        930        940        950
AAKRAGVTCI VLPAENKKDF YDLAAFITEG LEVHFVEHYR EIFDIAFPDE 

QAEALAVER
Length:959
Mass (Da):106,489
Last modified:December 1, 2000 - v2
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iB5E03D9C27C220FF
GO
Isoform 2 (identifier: P36776-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     42-105: Missing.

Show »
Length:895
Mass (Da):100,397
Checksum:iEF213C4E36C69AE1
GO
Isoform 3 (identifier: P36776-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-196: Missing.

Show »
Length:763
Mass (Da):85,642
Checksum:i3FEBB8DEEA651D7A
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
K7EKE6K7EKE6_HUMAN
Lon protease homolog, mitochondrial
LONP1
845Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7EJE8K7EJE8_HUMAN
Lon protease homolog, mitochondrial
LONP1
829Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7ERR6K7ERR6_HUMAN
Lon protease homolog, mitochondrial
LONP1
264Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7ER27K7ER27_HUMAN
Lon protease homolog, mitochondrial
LONP1
242Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7EQF8K7EQF8_HUMAN
Lon protease homolog, mitochondrial
LONP1
185Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7ER56K7ER56_HUMAN
Lon protease homolog, mitochondrial
LONP1
210Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
K7ERS1K7ERS1_HUMAN
Lon protease homolog, mitochondrial
LONP1
86Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the 'Sequence' section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence CAA52291 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti1 – 55MAAST…PWALW → MAGLWRRALATCDCGERRGA GCCGGRCWPRRGAGSHCSRS VVAPRPADLRRLSSLGTV in AAA61616 (PubMed:8248235).CuratedAdd BLAST55
Sequence conflicti65 – 66WR → CG in AAA61616 (PubMed:8248235).Curated2
Sequence conflicti257 – 258EL → DV in AAA61616 (PubMed:8248235).Curated2
Sequence conflicti423E → G in BAG51690 (PubMed:14702039).Curated1
Sequence conflicti456N → D in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti501I → V in BAG51690 (PubMed:14702039).Curated1
Sequence conflicti556A → T in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti842L → P in AAA61616 (PubMed:8248235).Curated1
Sequence conflicti859T → A in AAA61616 (PubMed:8248235).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05156487E → D. Corresponds to variant dbSNP:rs34413649EnsemblClinVar.1
Natural variantiVAR_051565241R → Q. Corresponds to variant dbSNP:rs11085147EnsemblClinVar.1
Natural variantiVAR_073338476E → A in CODASS. 1 Publication1
Natural variantiVAR_073339631S → Y in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255248EnsemblClinVar.1
Natural variantiVAR_073340670A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs770036526Ensembl.1
Natural variantiVAR_073341672R → C in CODASS. 1 PublicationCorresponds to variant dbSNP:rs777009012Ensembl.1
Natural variantiVAR_073342676P → S in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255247EnsemblClinVar.1
Natural variantiVAR_073343679R → H in CODASS. 1 PublicationCorresponds to variant dbSNP:rs549574673EnsemblClinVar.1
Natural variantiVAR_073344721R → G in CODASS. 1 PublicationCorresponds to variant dbSNP:rs147588238Ensembl.1
Natural variantiVAR_073345724A → V in CODASS. 1 PublicationCorresponds to variant dbSNP:rs879255249EnsemblClinVar.1
Natural variantiVAR_073346749P → S in CODASS. 1 Publication1
Natural variantiVAR_073347767G → E in CODASS. 1 PublicationCorresponds to variant dbSNP:rs562553348Ensembl.1
Natural variantiVAR_067708829A → T. Corresponds to variant dbSNP:rs35804229Ensembl.1
Natural variantiVAR_067709911V → I2 PublicationsCorresponds to variant dbSNP:rs1062373Ensembl.1
Natural variantiVAR_073348927Missing in CODASS. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0553101 – 196Missing in isoform 3. 1 PublicationAdd BLAST196
Alternative sequenceiVSP_05461742 – 105Missing in isoform 2. 1 PublicationAdd BLAST64

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...DDBJi
Links Updated
U02389 mRNA Translation: AAA61616.1
HQ204946 Genomic DNA Translation: ADP90374.1
HQ204947 Genomic DNA Translation: ADP90375.1
HQ204948 Genomic DNA Translation: ADP90376.1
HQ204949 Genomic DNA Translation: ADP90377.1
HQ204950 Genomic DNA Translation: ADP90378.1
HQ204951 Genomic DNA Translation: ADP90379.1
HQ204952 Genomic DNA Translation: ADP90380.1
HQ204953 Genomic DNA Translation: ADP90381.1
HQ204954 Genomic DNA Translation: ADP90382.1
HQ204955 Genomic DNA Translation: ADP90383.1
HQ204956 Genomic DNA Translation: ADP90384.1
HQ204957 Genomic DNA Translation: ADP90385.1
HQ204958 Genomic DNA Translation: ADP90386.1
HQ204959 Genomic DNA Translation: ADP90387.1
HQ204960 Genomic DNA Translation: ADP90388.1
HQ204961 Genomic DNA Translation: ADP90389.1
HQ204962 Genomic DNA Translation: ADP90390.1
HQ204963 Genomic DNA Translation: ADP90391.1
HQ204964 Genomic DNA Translation: ADP90392.1
HQ204965 Genomic DNA Translation: ADP90393.1
HQ204966 Genomic DNA Translation: ADP90394.1
HQ204968 Genomic DNA Translation: ADP90396.1
HQ204969 Genomic DNA Translation: ADP90397.1
HQ204970 Genomic DNA Translation: ADP90398.1
HQ204971 Genomic DNA Translation: ADP90399.1
HQ204972 Genomic DNA Translation: ADP90400.1
HQ204973 Genomic DNA Translation: ADP90401.1
HQ204974 Genomic DNA Translation: ADP90402.1
HQ204975 Genomic DNA Translation: ADP90403.1
HQ204976 Genomic DNA Translation: ADP90404.1
HQ204977 Genomic DNA Translation: ADP90405.1
HQ204978 Genomic DNA Translation: ADP90406.1
HQ204979 Genomic DNA Translation: ADP90407.1
HQ204980 Genomic DNA Translation: ADP90408.1
HQ204981 Genomic DNA Translation: ADP90409.1
HQ204982 Genomic DNA Translation: ADP90410.1
HQ204983 Genomic DNA Translation: ADP90411.1
HQ204984 Genomic DNA Translation: ADP90412.1
HQ204985 Genomic DNA Translation: ADP90413.1
AF059309 AF059308 Genomic DNA Translation: AAD24414.1
AK056366 mRNA Translation: BAG51690.1
AK096626 mRNA Translation: BAC04829.1
AC011499 Genomic DNA No translation available.
CH471139 Genomic DNA Translation: EAW69151.1
CH471139 Genomic DNA Translation: EAW69154.1
BC000235 mRNA Translation: AAH00235.1
X74215 mRNA Translation: CAA52291.1 Different initiation.
X76040 mRNA Translation: CAA53625.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS12148.1 [P36776-1]
CCDS62507.1 [P36776-3]
CCDS62508.1 [P36776-2]

Protein sequence database of the Protein Information Resource

More...PIRi		S42366
S57342

NCBI Reference Sequences

More...RefSeqi		NP_001263408.1, NM_001276479.1 [P36776-2]
NP_001263409.1, NM_001276480.1 [P36776-3]
NP_004784.2, NM_004793.3 [P36776-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000360614; ENSP00000353826; ENSG00000196365 [P36776-1]
ENST00000540670; ENSP00000441523; ENSG00000196365 [P36776-3]
ENST00000593119; ENSP00000468541; ENSG00000196365 [P36776-2]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		9361

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:9361

UCSC genome browser

More...UCSCi		uc002mcx.5, human [P36776-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U02389 mRNA Translation: AAA61616.1
HQ204946 Genomic DNA Translation: ADP90374.1
HQ204947 Genomic DNA Translation: ADP90375.1
HQ204948 Genomic DNA Translation: ADP90376.1
HQ204949 Genomic DNA Translation: ADP90377.1
HQ204950 Genomic DNA Translation: ADP90378.1
HQ204951 Genomic DNA Translation: ADP90379.1
HQ204952 Genomic DNA Translation: ADP90380.1
HQ204953 Genomic DNA Translation: ADP90381.1
HQ204954 Genomic DNA Translation: ADP90382.1
HQ204955 Genomic DNA Translation: ADP90383.1
HQ204956 Genomic DNA Translation: ADP90384.1
HQ204957 Genomic DNA Translation: ADP90385.1
HQ204958 Genomic DNA Translation: ADP90386.1
HQ204959 Genomic DNA Translation: ADP90387.1
HQ204960 Genomic DNA Translation: ADP90388.1
HQ204961 Genomic DNA Translation: ADP90389.1
HQ204962 Genomic DNA Translation: ADP90390.1
HQ204963 Genomic DNA Translation: ADP90391.1
HQ204964 Genomic DNA Translation: ADP90392.1
HQ204965 Genomic DNA Translation: ADP90393.1
HQ204966 Genomic DNA Translation: ADP90394.1
HQ204968 Genomic DNA Translation: ADP90396.1
HQ204969 Genomic DNA Translation: ADP90397.1
HQ204970 Genomic DNA Translation: ADP90398.1
HQ204971 Genomic DNA Translation: ADP90399.1
HQ204972 Genomic DNA Translation: ADP90400.1
HQ204973 Genomic DNA Translation: ADP90401.1
HQ204974 Genomic DNA Translation: ADP90402.1
HQ204975 Genomic DNA Translation: ADP90403.1
HQ204976 Genomic DNA Translation: ADP90404.1
HQ204977 Genomic DNA Translation: ADP90405.1
HQ204978 Genomic DNA Translation: ADP90406.1
HQ204979 Genomic DNA Translation: ADP90407.1
HQ204980 Genomic DNA Translation: ADP90408.1
HQ204981 Genomic DNA Translation: ADP90409.1
HQ204982 Genomic DNA Translation: ADP90410.1
HQ204983 Genomic DNA Translation: ADP90411.1
HQ204984 Genomic DNA Translation: ADP90412.1
HQ204985 Genomic DNA Translation: ADP90413.1
AF059309 AF059308 Genomic DNA Translation: AAD24414.1
AK056366 mRNA Translation: BAG51690.1
AK096626 mRNA Translation: BAC04829.1
AC011499 Genomic DNA No translation available.
CH471139 Genomic DNA Translation: EAW69151.1
CH471139 Genomic DNA Translation: EAW69154.1
BC000235 mRNA Translation: AAH00235.1
X74215 mRNA Translation: CAA52291.1 Different initiation.
X76040 mRNA Translation: CAA53625.1
CCDSiCCDS12148.1 [P36776-1]
CCDS62507.1 [P36776-3]
CCDS62508.1 [P36776-2]
PIRiS42366
S57342
RefSeqiNP_001263408.1, NM_001276479.1 [P36776-2]
NP_001263409.1, NM_001276480.1 [P36776-3]
NP_004784.2, NM_004793.3 [P36776-1]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2X36X-ray2.00A/B/C/D/E/F753-959[»]
7KRZelectron microscopy3.20A/B/C/D/E/F414-947[»]
7KSLelectron microscopy3.40A/B/C/E/F421-947[»]
7KSMelectron microscopy3.20A/B/C/D/E/F416-947[»]
7NFYelectron microscopy3.90A/B/C/D/E/F115-959[»]
7NG4electron microscopy4.40A/B/C/D/E/F115-959[»]
7NG5electron microscopy3.80A/B/C/D/E/F115-959[»]
SMRiP36776
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi114762, 293 interactors
IntActiP36776, 76 interactors
MINTiP36776
STRINGi9606.ENSP00000353826

Protein family/group databases

MEROPSiS16.002
MoonDBiP36776, Curated

PTM databases

iPTMnetiP36776
MetOSiteiP36776
PhosphoSitePlusiP36776
SwissPalmiP36776

Genetic variation databases

BioMutaiLONP1
DMDMi12644239

Proteomic databases

EPDiP36776
jPOSTiP36776
MassIVEiP36776
MaxQBiP36776
PaxDbiP36776
PeptideAtlasiP36776
PRIDEiP36776
ProteomicsDBi24918
55221 [P36776-1]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		811, 287 antibodies

The DNASU plasmid repository

More...DNASUi		9361

Genome annotation databases

EnsembliENST00000360614; ENSP00000353826; ENSG00000196365 [P36776-1]
ENST00000540670; ENSP00000441523; ENSG00000196365 [P36776-3]
ENST00000593119; ENSP00000468541; ENSG00000196365 [P36776-2]
GeneIDi9361
KEGGihsa:9361
UCSCiuc002mcx.5, human [P36776-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		9361
DisGeNETi9361

GeneCards: human genes, protein and diseases

More...GeneCardsi		LONP1
HGNCiHGNC:9479, LONP1
HPAiENSG00000196365, Tissue enhanced (adrenal)
MalaCardsiLONP1
MIMi600373, phenotype
605490, gene
neXtProtiNX_P36776
OpenTargetsiENSG00000196365
Orphaneti1458, CODAS syndrome
79243, Pyruvate dehydrogenase E1-alpha deficiency
PharmGKBiPA162394145
VEuPathDBiHostDB:ENSG00000196365.11

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG2004, Eukaryota
GeneTreeiENSGT00530000063553
HOGENOMiCLU_004109_1_1_1
InParanoidiP36776
OMAiENVAHED
OrthoDBi528132at2759
PhylomeDBiP36776
TreeFamiTF105001

Enzyme and pathway databases

BRENDAi3.4.21.53, 2681
PathwayCommonsiP36776

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		9361, 762 hits in 992 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		LONP1, human
EvolutionaryTraceiP36776

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		LONP1

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		9361
PharosiP36776, Tbio

Protein Ontology

More...PROi		PR:P36776
RNActiP36776, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000196365, Expressed in right adrenal gland and 233 other tissues
ExpressionAtlasiP36776, baseline and differential
GenevisibleiP36776, HS

Family and domain databases

Gene3Di3.30.230.10, 1 hit
HAMAPiMF_03120, lonm_euk, 1 hit
InterProiView protein in InterPro
IPR003593, AAA+_ATPase
IPR003959, ATPase_AAA_core
IPR004815, Lon_bac/euk-typ
IPR008269, Lon_proteolytic
IPR027065, Lon_Prtase
IPR003111, Lon_substr-bd
IPR027503, Lonm_euk
IPR027417, P-loop_NTPase
IPR008268, Peptidase_S16_AS
IPR015947, PUA-like_sf
IPR020568, Ribosomal_S5_D2-typ_fold
IPR014721, Ribosomal_S5_D2-typ_fold_subgr
PANTHERiPTHR43718, PTHR43718, 1 hit
PfamiView protein in Pfam
PF00004, AAA, 1 hit
PF05362, Lon_C, 1 hit
PF02190, LON_substr_bdg, 1 hit
SMARTiView protein in SMART
SM00382, AAA, 1 hit
SM00464, LON, 1 hit
SUPFAMiSSF52540, SSF52540, 1 hit
SSF54211, SSF54211, 1 hit
SSF88697, SSF88697, 1 hit
TIGRFAMsiTIGR00763, lon, 1 hit
PROSITEiView protein in PROSITE
PS51787, LON_N, 1 hit
PS51786, LON_PROTEOLYTIC, 1 hit
PS01046, LON_SER, 1 hit

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiLONM_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P36776
Secondary accession number(s): B3KPH8
, D6W635, E5KMH8, F5GZ27, P36777, Q8N8K8, Q9UQ95
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: December 1, 2000
Last modified: June 2, 2021
This is version 205 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Reference proteome

Documents

  1. Peptidase families
    Classification of peptidase families and list of entries
  2. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  3. Human entries with genetic variants
    List of human entries with genetic variants
  4. Human variants curated from literature reports
    Index of human variants curated from literature reports
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families
UniProt is an ELIXIR core data resource
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