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Protein

Presequence protease, mitochondrial

Gene

PITRM1

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

Metalloendopeptidase of the mitochondrial matrix that functions in peptide cleavage and degradation rather than in protein processing (PubMed:10360838, PubMed:16849325, PubMed:19196155, PubMed:24931469). Has an ATP-independent activity (PubMed:16849325). Specifically cleaves peptides in the range of 5 to 65 residues (PubMed:19196155). Shows a preference for cleavage after small polar residues and before basic residues, but without any positional preference (PubMed:10360838, PubMed:19196155, PubMed:24931469). Degrades the transit peptides of mitochondrial proteins after their cleavage (PubMed:19196155). Also degrades other unstructured peptides (PubMed:19196155). It is also able to degrade amyloid-beta protein 40, one of the peptides produced by APP processing, when it accumulates in mitochondrion (PubMed:16849325, PubMed:24931469). It is a highly efficient protease, at least toward amyloid-beta protein 40 (PubMed:24931469). Cleaves that peptide at a specific position and is probably not processive, releasing digested peptides intermediates that can be further cleaved subsequently (PubMed:24931469).4 Publications

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Zn2+1 PublicationNote: Binds 1 zinc ion per subunit.1 Publication

<p>This subsection of the ‘Function’ 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

Mainly exists in a closed and catalytically competent conformation but a closed-to-open switch allows substrate entry into the catalytic chamber (PubMed:24931469). Substrate binding induces closure and dimerization (PubMed:24931469). A disulfide bond may lock the enzyme in a closed conformation preventing substrate entry into the catalytic chamber, participating in redox regulation of the enzyme (Probable). Inhibited by metal-chelating agents (PubMed:10360838). Inhibited by nickel and zinc excess, and slightly activated by manganese (PubMed:19196155).2 Publications3 Publications

<p>This subsection of the ‘Function’ section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=1.07 µM for leumorphin ARG-ARG-GLN-PHE-LYS-VAL-VAL-THR-ARG-SER-GLN peptide (at pH 7.5)1 Publication
  2. KM=0.5 µM for TYR-GLY-GLY-LEU-ARG-ARG-GLY-GLN peptide (at pH 7.5)1 Publication

    pH dependencei

    Optimum pH is 7.7.1 Publication

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi104Zinc; via tele nitrogen; catalyticCombined sources1 Publication1
    <p>This subsection of the ‘Function’ 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 sitei107Proton acceptor2 Publications1
    Metal bindingi108Zinc; via tele nitrogen; catalyticCombined sources1 Publication1
    Metal bindingi205Zinc; catalyticCombined sources1 Publication1

    <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

    • enzyme activator activity Source: ProtInc
    • metalloendopeptidase activity Source: UniProtKB
    • metallopeptidase activity Source: Reactome
    • zinc ion binding Source: UniProtKB

    GO - Biological processi

    <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 functionHydrolase, Metalloprotease, Protease
    LigandMetal-binding, Zinc

    Enzyme and pathway databases

    Reactome - a knowledgebase of biological pathways and processes

    More...
    Reactomei
    R-HSA-1268020 Mitochondrial protein import

    SignaLink: a signaling pathway resource with multi-layered regulatory networks

    More...
    SignaLinki
    Q5JRX3

    Protein family/group databases

    MEROPS protease database

    More...
    MEROPSi
    M16.009

    <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_and_taxonomy_section">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:
    Presequence protease, mitochondrial1 Publication (EC:3.4.24.-4 Publications)
    Short name:
    hPreP1 Publication
    Alternative name(s):
    Pitrilysin metalloproteinase 11 Publication
    Short name:
    Metalloprotease 11 Publication
    Short name:
    hMP11 Publication
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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:PITRM1Imported
    Synonyms:KIAA1104Imported, MP11 Publication
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_manual">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 10

    Organism-specific databases

    Eukaryotic Pathogen Database Resources

    More...
    EuPathDBi
    HostDB:ENSG00000107959.15

    Human Gene Nomenclature Database

    More...
    HGNCi
    HGNC:17663 PITRM1

    neXtProt; the human protein knowledge platform

    More...
    neXtProti
    NX_Q5JRX3

    <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

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    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

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'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>Mutagenesisi1 – 15Missing : Loss of localization to the mitochondrion. 1 PublicationAdd BLAST15
    Mutagenesisi107E → Q: Loss of metalloendopeptidase activity. 2 Publications1
    Mutagenesisi119C → S: No loss of metalloendopeptidase activity under oxidizing conditions. 1 Publication1
    Mutagenesisi557L → E: Decreased metalloendopeptidase activity without effect on protein stability. 1 Publication1
    Mutagenesisi558P → G: Decreased metalloendopeptidase activity without effect on protein stability. 1 Publication1

    Organism-specific databases

    DisGeNET

    More...
    DisGeNETi
    10531

    Open Targets

    More...
    OpenTargetsi
    ENSG00000107959

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...
    PharmGKBi
    PA134902269

    Chemistry databases

    ChEMBL database of bioactive drug-like small molecules

    More...
    ChEMBLi
    CHEMBL3124731

    Polymorphism and mutation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...
    BioMutai
    PITRM1

    Domain mapping of disease mutations (DMDM)

    More...
    DMDMi
    485956568

    <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 – 28MitochondrionSequence analysisAdd BLAST28
    <p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000024993129 – 1037Presequence protease, mitochondrialAdd BLAST1009

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi119 ↔ 5561 Publication
    <p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei759N6-acetyllysineBy similarity1
    Modified residuei770N6-acetyllysine; alternateBy similarity1
    Modified residuei770N6-succinyllysine; alternateBy similarity1
    Modified residuei849N6-succinyllysineBy similarity1
    Modified residuei884N6-acetyllysineBy similarity1
    Modified residuei946N6-succinyllysineBy similarity1

    <p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

    A disulfide bond locks the enzyme in the closed conformation preventing substrate entry into the catalytic chamber.2 Publications

    Keywords - PTMi

    Acetylation, Disulfide bond

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...
    EPDi
    Q5JRX3

    MaxQB - The MaxQuant DataBase

    More...
    MaxQBi
    Q5JRX3

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

    More...
    PaxDbi
    Q5JRX3

    PeptideAtlas

    More...
    PeptideAtlasi
    Q5JRX3

    PRoteomics IDEntifications database

    More...
    PRIDEi
    Q5JRX3

    ProteomicsDB human proteome resource

    More...
    ProteomicsDBi
    63119
    63120 [Q5JRX3-2]

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...
    iPTMneti
    Q5JRX3

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

    More...
    PhosphoSitePlusi
    Q5JRX3

    SwissPalm database of S-palmitoylation events

    More...
    SwissPalmi
    Q5JRX3

    <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

    Widely expressed. Expressed at higher level in muscle and heart compared to brain, pancreas, liver, lung and placenta.1 Publication

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...
    Bgeei
    ENSG00000107959 Expressed in 226 organ(s), highest expression level in left lobe of thyroid gland

    CleanEx database of gene expression profiles

    More...
    CleanExi
    HS_PITRM1

    ExpressionAtlas, Differential and Baseline Expression

    More...
    ExpressionAtlasi
    Q5JRX3 baseline and differential

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...
    Genevisiblei
    Q5JRX3 HS

    Organism-specific databases

    Human Protein Atlas

    More...
    HPAi
    HPA006753
    HPA006754

    <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_section">'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_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Monomer and homodimer; homodimerization is induced by binding of the substrate.1 Publication

    <p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">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="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

    WithEntry#Exp.IntActNotes
    APPP050673EBI-16109799,EBI-2431589

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGrid)

    More...
    BioGridi
    115786, 79 interactors

    Database of interacting proteins

    More...
    DIPi
    DIP-52900N

    Protein interaction database and analysis system

    More...
    IntActi
    Q5JRX3, 10 interactors

    STRING: functional protein association networks

    More...
    STRINGi
    9606.ENSP00000370377

    Chemistry databases

    BindingDB database of measured binding affinities

    More...
    BindingDBi
    Q5JRX3

    <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

    11037
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

    More...
    ProteinModelPortali
    Q5JRX3

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

    More...
    SMRi
    Q5JRX3

    Database of comparative protein structure models

    More...
    ModBasei
    Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...
    MobiDBi
    Search...

    <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

    <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 M16 family. PreP subfamily.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...
    eggNOGi
    KOG2019 Eukaryota
    COG1026 LUCA

    Ensembl GeneTree

    More...
    GeneTreei
    ENSGT00390000018381

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...
    HOGENOMi
    HOG000008829

    The HOVERGEN Database of Homologous Vertebrate Genes

    More...
    HOVERGENi
    HBG082167

    InParanoid: Eukaryotic Ortholog Groups

    More...
    InParanoidi
    Q5JRX3

    KEGG Orthology (KO)

    More...
    KOi
    K06972

    Identification of Orthologs from Complete Genome Data

    More...
    OMAi
    FTYGNFP

    Database of Orthologous Groups

    More...
    OrthoDBi
    EOG091G0108

    TreeFam database of animal gene trees

    More...
    TreeFami
    TF300333

    Family and domain databases

    Integrated resource of protein families, domains and functional sites

    More...
    InterProi
    View protein in InterPro
    IPR011249 Metalloenz_LuxS/M16
    IPR011765 Pept_M16_N
    IPR007863 Peptidase_M16_C
    IPR013578 Peptidase_M16C_assoc

    Pfam protein domain database

    More...
    Pfami
    View protein in Pfam
    PF08367 M16C_assoc, 1 hit
    PF00675 Peptidase_M16, 1 hit
    PF05193 Peptidase_M16_C, 2 hits

    Simple Modular Architecture Research Tool; a protein domain database

    More...
    SMARTi
    View protein in SMART
    SM01264 M16C_associated, 1 hit

    Superfamily database of structural and functional annotation

    More...
    SUPFAMi
    SSF63411 SSF63411, 4 hits

    <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_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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 5 potential isoforms that are computationally mapped.Show allAlign All

    Isoform 1 (identifier: Q5JRX3-1) [UniParc]FASTAAdd to basket

    This isoform has been chosen as the 'canonical' 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
    MWRCGGRQGL CVLRRLSGGH AHHRAWRWNS NRACERALQY KLGDKIHGFT
    60 70 80 90 100
    VNQVTSVPEL FLTAVKLTHD DTGARYLHLA REDTNNLFSV QFRTTPMDST
    110 120 130 140 150
    GVPHILEHTV LCGSQKYPCR DPFFKMLNRS LSTFMNAFTA SDYTLYPFST
    160 170 180 190 200
    QNPKDFQNLL SVYLDATFFP CLRELDFWQE GWRLEHENPS DPQTPLVFKG
    210 220 230 240 250
    VVFNEMKGAF TDNERIFSQH LQNRLLPDHT YSVVSGGDPL CIPELTWEQL
    260 270 280 290 300
    KQFHATHYHP SNARFFTYGN FPLEQHLKQI HEEALSKFQK IEPSTVVPAQ
    310 320 330 340 350
    TPWDKPREFQ ITCGPDSFAT DPSKQTTISV SFLLPDITDT FEAFTLSLLS
    360 370 380 390 400
    SLLTSGPNSP FYKALIESGL GTDFSPDVGY NGYTREAYFS VGLQGIAEKD
    410 420 430 440 450
    IETVRSLIDR TIDEVVEKGF EDDRIEALLH KIEIQMKHQS TSFGLMLTSY
    460 470 480 490 500
    IASCWNHDGD PVELLKLGNQ LAKFRQCLQE NPKFLQEKVK QYFKNNQHKL
    510 520 530 540 550
    TLSMRPDDKY HEKQAQVEAT KLKQKVEALS PGDRQQIYEK GLELRSQQSK
    560 570 580 590 600
    PQDASCLPAL KVSDIEPTIP VTELDVVLTA GDIPVQYCAQ PTNGMVYFRA
    610 620 630 640 650
    FSSLNTLPEE LRPYVPLFCS VLTKLGCGLL DYREQAQQIE LKTGGMSASP
    660 670 680 690 700
    HVLPDDSHMD TYEQGVLFSS LCLDRNLPDM MQLWSEIFNN PCFEEEEHFK
    710 720 730 740 750
    VLVKMTAQEL ANGIPDSGHL YASIRAGRTL TPAGDLQETF SGMDQVRLMK
    760 770 780 790 800
    RIAEMTDIKP ILRKLPRIKK HLLNGDNMRC SVNATPQQMP QTEKAVEDFL
    810 820 830 840 850
    RSIGRSKKER RPVRPHTVEK PVPSSSGGDA HVPHGSQVIR KLVMEPTFKP
    860 870 880 890 900
    WQMKTHFLMP FPVNYVGECI RTVPYTDPDH ASLKILARLM TAKFLHTEIR
    910 920 930 940 950
    EKGGAYGGGA KLSHNGIFTL YSYRDPNTIE TLQSFGKAVD WAKSGKFTQQ
    960 970 980 990 1000
    DIDEAKLSVF STVDAPVAPS DKGMDHFLYG LSDEMKQAHR EQLFAVSHDK
    1010 1020 1030
    LLAVSDRYLG TGKSTHGLAI LGPENPKIAK DPSWIIQ
    Length:1,037
    Mass (Da):117,413
    Last modified:May 1, 2013 - v3
    <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:iF488389F0E219718
    GO
    Isoform 2 (identifier: Q5JRX3-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         664-664: Q → QV

    Note: No experimental confirmation available.
    Show »
    Length:1,038
    Mass (Da):117,512
    Checksum:i8A55B3A43B434A4E
    GO
    Isoform 3 (identifier: Q5JRX3-3) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-53: MWRCGGRQGLCVLRRLSGGHAHHRAWRWNSNRACERALQYKLGDKIHGFTVNQ → MRNVALRRAAGPVCAEAAERR
         624-689: Missing.

    Show »
    Length:939
    Mass (Da):106,065
    Checksum:i21E09CEE32236867
    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 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    Gene namesLengthAnnotation
    A0A0A0MRX9A0A0A0MRX9_HUMAN
    Presequence protease, mitochondrial
    PITRM1
    595Annotation 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>
    H0Y4F7H0Y4F7_HUMAN
    Presequence protease, mitochondrial
    PITRM1
    233Annotation 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>
    H0Y7L7H0Y7L7_HUMAN
    Presequence protease, mitochondrial
    PITRM1
    259Annotation 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>
    B1APQ0B1APQ0_HUMAN
    Presequence protease, mitochondrial
    PITRM1
    184Annotation 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>
    B1APQ1B1APQ1_HUMAN
    Presequence protease, mitochondrial
    PITRM1
    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 AAH01150 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
    The sequence CAI39997 differs from that shown. Reason: Erroneous gene model prediction.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 conflicti121D → N in AAC67244 (PubMed:10360838).Curated1
    Sequence conflicti211T → V in CAI40001 (PubMed:15164054).Curated1
    Sequence conflicti212D → N in CAI40001 (PubMed:15164054).Curated1
    Sequence conflicti373D → E in AAC67244 (PubMed:10360838).Curated1
    Sequence conflicti418 – 420KGF → TRI in AAC67244 (PubMed:10360838).Curated3
    Sequence conflicti883 – 884LK → FE in AAH95422 (PubMed:15489334).Curated2

    Natural variant

    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_0275178Q → R1 PublicationCorresponds to variant dbSNP:rs11818724Ensembl.1
    Natural variantiVAR_027518145L → V1 PublicationCorresponds to variant dbSNP:rs9423502Ensembl.1
    Natural variantiVAR_027519169F → S1 PublicationCorresponds to variant dbSNP:rs3814596Ensembl.1
    Natural variantiVAR_027520328I → V3 PublicationsCorresponds to variant dbSNP:rs4242746Ensembl.1
    Natural variantiVAR_027521397A → V3 PublicationsCorresponds to variant dbSNP:rs3182535Ensembl.1
    Natural variantiVAR_027522516Q → H. Corresponds to variant dbSNP:rs3765101Ensembl.1
    Natural variantiVAR_057059554A → D. Corresponds to variant dbSNP:rs12248937Ensembl.1
    Natural variantiVAR_027523621V → I1 PublicationCorresponds to variant dbSNP:rs2388556Ensembl.1
    Natural variantiVAR_057060805R → Q. Corresponds to variant dbSNP:rs34837384Ensembl.1
    Natural variantiVAR_027524952I → M. Corresponds to variant dbSNP:rs2279219Ensembl.1
    Natural variantiVAR_027525963V → I1 PublicationCorresponds to variant dbSNP:rs17849904Ensembl.1
    Natural variantiVAR_027526969P → L. Corresponds to variant dbSNP:rs2279218Ensembl.1
    Natural variantiVAR_0275271037Q → RCombined sources4 PublicationsCorresponds to variant dbSNP:rs6901Ensembl.1

    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. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0464941 – 53MWRCG…FTVNQ → MRNVALRRAAGPVCAEAAER R in isoform 3. 1 PublicationAdd BLAST53
    Alternative sequenceiVSP_046495624 – 689Missing in isoform 3. 1 PublicationAdd BLAST66
    Alternative sequenceiVSP_020597664Q → QV in isoform 2. 1 Publication1

    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
    AF061243 mRNA Translation: AAC67244.1
    AK002061 mRNA Translation: BAG51008.1
    AK303406 mRNA Translation: BAG64460.1
    AL451164 Genomic DNA Translation: CAI40001.1
    AL451164 Genomic DNA Translation: CAI39997.1 Sequence problems.
    BC001150 mRNA Translation: AAH01150.1 Different initiation.
    BC005025 mRNA Translation: AAH05025.1
    BC095422 mRNA Translation: AAH95422.1
    BC111987 mRNA Translation: AAI11988.1
    BC113369 mRNA Translation: AAI13370.1
    AB029027 mRNA Translation: BAA83056.2

    The Consensus CDS (CCDS) project

    More...
    CCDSi
    CCDS55699.1 [Q5JRX3-2]
    CCDS55700.1 [Q5JRX3-3]
    CCDS59208.1 [Q5JRX3-1]

    NCBI Reference Sequences

    More...
    RefSeqi
    NP_001229236.1, NM_001242307.1 [Q5JRX3-2]
    NP_001229238.1, NM_001242309.1 [Q5JRX3-3]
    NP_055704.2, NM_014889.3 [Q5JRX3-1]

    UniGene gene-oriented nucleotide sequence clusters

    More...
    UniGenei
    Hs.528300

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...
    Ensembli
    ENST00000224949; ENSP00000224949; ENSG00000107959 [Q5JRX3-1]
    ENST00000380989; ENSP00000370377; ENSG00000107959 [Q5JRX3-2]
    ENST00000451104; ENSP00000401201; ENSG00000107959 [Q5JRX3-3]

    Database of genes from NCBI RefSeq genomes

    More...
    GeneIDi
    10531

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...
    KEGGi
    hsa:10531

    UCSC genome browser

    More...
    UCSCi
    uc001igt.3 human [Q5JRX3-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    <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
    AF061243 mRNA Translation: AAC67244.1
    AK002061 mRNA Translation: BAG51008.1
    AK303406 mRNA Translation: BAG64460.1
    AL451164 Genomic DNA Translation: CAI40001.1
    AL451164 Genomic DNA Translation: CAI39997.1 Sequence problems.
    BC001150 mRNA Translation: AAH01150.1 Different initiation.
    BC005025 mRNA Translation: AAH05025.1
    BC095422 mRNA Translation: AAH95422.1
    BC111987 mRNA Translation: AAI11988.1
    BC113369 mRNA Translation: AAI13370.1
    AB029027 mRNA Translation: BAA83056.2
    CCDSiCCDS55699.1 [Q5JRX3-2]
    CCDS55700.1 [Q5JRX3-3]
    CCDS59208.1 [Q5JRX3-1]
    RefSeqiNP_001229236.1, NM_001242307.1 [Q5JRX3-2]
    NP_001229238.1, NM_001242309.1 [Q5JRX3-3]
    NP_055704.2, NM_014889.3 [Q5JRX3-1]
    UniGeneiHs.528300

    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
    4L3TX-ray2.03A/B33-1037[»]
    4NGEX-ray2.70A/D33-1037[»]
    4RPUX-ray2.27A/B33-1037[»]
    ProteinModelPortaliQ5JRX3
    SMRiQ5JRX3
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi115786, 79 interactors
    DIPiDIP-52900N
    IntActiQ5JRX3, 10 interactors
    STRINGi9606.ENSP00000370377

    Chemistry databases

    BindingDBiQ5JRX3
    ChEMBLiCHEMBL3124731

    Protein family/group databases

    MEROPSiM16.009

    PTM databases

    iPTMnetiQ5JRX3
    PhosphoSitePlusiQ5JRX3
    SwissPalmiQ5JRX3

    Polymorphism and mutation databases

    BioMutaiPITRM1
    DMDMi485956568

    Proteomic databases

    EPDiQ5JRX3
    MaxQBiQ5JRX3
    PaxDbiQ5JRX3
    PeptideAtlasiQ5JRX3
    PRIDEiQ5JRX3
    ProteomicsDBi63119
    63120 [Q5JRX3-2]

    Protocols and materials databases

    The DNASU plasmid repository

    More...
    DNASUi
    10531
    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENST00000224949; ENSP00000224949; ENSG00000107959 [Q5JRX3-1]
    ENST00000380989; ENSP00000370377; ENSG00000107959 [Q5JRX3-2]
    ENST00000451104; ENSP00000401201; ENSG00000107959 [Q5JRX3-3]
    GeneIDi10531
    KEGGihsa:10531
    UCSCiuc001igt.3 human [Q5JRX3-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...
    CTDi
    10531
    DisGeNETi10531
    EuPathDBiHostDB:ENSG00000107959.15

    GeneCards: human genes, protein and diseases

    More...
    GeneCardsi
    PITRM1

    H-Invitational Database, human transcriptome db

    More...
    H-InvDBi
    HIX0008597
    HGNCiHGNC:17663 PITRM1
    HPAiHPA006753
    HPA006754
    neXtProtiNX_Q5JRX3
    OpenTargetsiENSG00000107959
    PharmGKBiPA134902269

    Human Unidentified Gene-Encoded large proteins database

    More...
    HUGEi
    Search...

    GenAtlas: human gene database

    More...
    GenAtlasi
    Search...

    Phylogenomic databases

    eggNOGiKOG2019 Eukaryota
    COG1026 LUCA
    GeneTreeiENSGT00390000018381
    HOGENOMiHOG000008829
    HOVERGENiHBG082167
    InParanoidiQ5JRX3
    KOiK06972
    OMAiFTYGNFP
    OrthoDBiEOG091G0108
    TreeFamiTF300333

    Enzyme and pathway databases

    ReactomeiR-HSA-1268020 Mitochondrial protein import
    SignaLinkiQ5JRX3

    Miscellaneous databases

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

    More...
    ChiTaRSi
    PITRM1 human

    The Gene Wiki collection of pages on human genes and proteins

    More...
    GeneWikii
    PITRM1

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

    More...
    GenomeRNAii
    10531

    Protein Ontology

    More...
    PROi
    PR:Q5JRX3

    Gene expression databases

    BgeeiENSG00000107959 Expressed in 226 organ(s), highest expression level in left lobe of thyroid gland
    CleanExiHS_PITRM1
    ExpressionAtlasiQ5JRX3 baseline and differential
    GenevisibleiQ5JRX3 HS

    Family and domain databases

    InterProiView protein in InterPro
    IPR011249 Metalloenz_LuxS/M16
    IPR011765 Pept_M16_N
    IPR007863 Peptidase_M16_C
    IPR013578 Peptidase_M16C_assoc
    PfamiView protein in Pfam
    PF08367 M16C_assoc, 1 hit
    PF00675 Peptidase_M16, 1 hit
    PF05193 Peptidase_M16_C, 2 hits
    SMARTiView protein in SMART
    SM01264 M16C_associated, 1 hit
    SUPFAMiSSF63411 SSF63411, 4 hits

    ProtoNet; Automatic hierarchical classification of proteins

    More...
    ProtoNeti
    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 nameiPREP_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: Q5JRX3
    Secondary accession number(s): B3KMJ6
    , B4E0J8, C9JSL2, E7ES23, O95204, Q2M2G6, Q4VBR1, Q5JRW7, Q7L5Z7, Q9BSI6, Q9BVJ5, Q9UPP8
    <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_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 19, 2006
    Last sequence update: May 1, 2013
    Last modified: December 5, 2018
    This is version 134 of the entry and version 3 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, Complete proteome, Reference proteome

    Documents

    1. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    2. SIMILARITY comments
      Index of protein domains and families
    3. Human chromosome 10
      Human chromosome 10: entries, gene names and cross-references to MIM
    4. Peptidase families
      Classification of peptidase families and list of entries
    5. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    6. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
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