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Protein

Ubiquitin carboxyl-terminal hydrolase isozyme L1

Gene

UCHL1

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

Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity.2 Publications

Miscellaneous

Oxidation of Met-1, Met-6, Met-12, Met-124 and Met-179 to methionine sulfoxide, and oxidation of Cys-220 to cysteine sulfonic acid have been observed in brains from Alzheimer disease (AD) and Parkinson disease (PD) patients. In AD, UCHL1 was found to be associated with neurofibrillary tangles. In contrast to UCHL3, does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8.

Caution

PubMed:9774100 reports the association of mutation Ile93Met with Parkinson disease. However, according to PubMed:16450370 this association is uncertain and UCHL1 is not a susceptibility gene for Parkinson disease.Curated
The oxidation forms of Met-1, Met-6, Met-12, Met-124, Met-179 and Cys-220 are subject of controversy and could be the artifactual results of sample handling.1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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

  • Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).1 Publication EC:3.4.19.12

<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=122 nM for Ub-AMC3 Publications
  2. KM=1.20 µM for ubiquitin ethyl ester3 Publications
  1. Vmax=0.47 µmol/min/mg enzyme toward Ub-AMC3 Publications
  2. Vmax=25 µmol/min/mg enzyme toward ubiquitin ethyl ester3 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei1Susceptible to oxidation1 Publication1
Sitei6Susceptible to oxidation1 Publication1
Sitei12Susceptible to oxidation1 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 sitei90Nucleophile1 Publication1
Sitei124Susceptible to oxidation1 Publication1
Active sitei161Proton donor1 Publication1
Sitei176Important for enzyme activity1 Publication1
Sitei179Susceptible to oxidation1 Publication1
Sitei220Susceptible to oxidation1 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

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, Ligase, Protease, Thiol protease
Biological processUbl conjugation pathway

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
3.4.19.12 2681

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-5689603 UCH proteinases

SABIO-RK: Biochemical Reaction Kinetics Database

More...
SABIO-RKi
P09936

SIGNOR Signaling Network Open Resource

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SIGNORi
P09936

Protein family/group databases

MEROPS protease database

More...
MEROPSi
C12.001

<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:
Ubiquitin carboxyl-terminal hydrolase isozyme L1 (EC:3.4.19.12, EC:6.-.-.-)
Short name:
UCH-L1
Alternative name(s):
Neuron cytoplasmic protein 9.5
PGP 9.5
Short name:
PGP9.5
Ubiquitin thioesterase L1
<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:UCHL1
<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 4

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...
EuPathDBi
HostDB:ENSG00000154277.12

Human Gene Nomenclature Database

More...
HGNCi
HGNC:12513 UCHL1

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
191342 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P09936

<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

Cytoplasm, Endoplasmic reticulum, Membrane

<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

Parkinson disease 5 (PARK5)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA complex neurodegenerative disorder with manifestations ranging from typical Parkinson disease to dementia with Lewy bodies. Clinical features include parkinsonian symptoms (resting tremor, rigidity, postural instability and bradykinesia), dementia, diffuse Lewy body pathology, autonomic dysfunction, hallucinations and paranoia.
See also OMIM:613643
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_01567893I → M in PARK5; impaired enzymatic hydrolase activity; has about a 50% reduction in catalytic activity compared to wild-type protein. 4 PublicationsCorresponds to variant dbSNP:rs121917767EnsemblClinVar.1
Spastic paraplegia 79, autosomal recessive (SPG79)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG79 is characterized by childhood onset blindness, cerebellar ataxia, nystagmus, dorsal column dysfunction, and spasticity with upper motor neuron dysfunction.
See also OMIM:615491
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0708757E → A in SPG79; has decreased binding to ubiquitin and significantly decreased hydrolase activity compared to wild-type. 1 PublicationCorresponds to variant dbSNP:rs397515634EnsemblClinVar.1
Natural variantiVAR_078119178R → Q in SPG79; increased hydrolase activity; decreased protein abundance. 1 PublicationCorresponds to variant dbSNP:rs768996179EnsemblClinVar.1
Natural variantiVAR_078120216A → D in SPG79; decreased protein abundance. 1 PublicationCorresponds to variant dbSNP:rs1057519600Ensembl.1

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>Mutagenesisi73Q → R: No effect on enzymatic parameters. 1 Publication1
Mutagenesisi90C → S: Abolishes enzymatic activity. 3 Publications1
Mutagenesisi97H → Q or N: 2-fold increase in affinity for ubiquitin ethyl ester, slight reduction in enzymatic activity. 1 Publication1
Mutagenesisi161H → D: 10000-fold decrease in enzymatic activity; no change in affinity for ubiquitin ethyl ester. 1 Publication1
Mutagenesisi161H → K, Q, N or Y: Abolishes enzymatic activity. 1 Publication1
Mutagenesisi176D → N: 6-fold decrease in affinity for ubiquitin ethyl ester; 97.5% decrease in enzymatic activity. 1 Publication1
Mutagenesisi204F → A: Almost complete loss of activity. 1 Publication1

Keywords - Diseasei

Disease mutation, Hereditary spastic paraplegia, Neurodegeneration, Parkinson disease, Parkinsonism

Organism-specific databases

DisGeNET

More...
DisGeNETi
7345

MalaCards human disease database

More...
MalaCardsi
UCHL1
MIMi613643 phenotype
615491 phenotype

Open Targets

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OpenTargetsi
ENSG00000154277

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

More...
Orphaneti
352654 Early-onset progressive neurodegeneration-blindness-ataxia-spasticity syndrome
2828 Young-onset Parkinson disease

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA37160

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

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ChEMBLi
CHEMBL6159

IUPHAR/BPS Guide to PHARMACOLOGY

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GuidetoPHARMACOLOGYi
2426

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
UCHL1

Domain mapping of disease mutations (DMDM)

More...
DMDMi
136681

<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 polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00002110551 – 220Ubiquitin carboxyl-terminal hydrolase isozyme L1Add BLAST220
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section describes a propeptide, which is a part of a protein that is cleaved during maturation or activation. Once cleaved, a propeptide generally has no independent biological function.<p><a href='/help/propep' target='_top'>More...</a></p>PropeptideiPRO_0000414311221 – 223Removed in mature formCurated3

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<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 residuei125PhosphoserineBy similarity1
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section specifies the position(s) and the type of covalently attached lipid group(s).<p><a href='/help/lipid' target='_top'>More...</a></p>Lipidationi220S-farnesyl cysteine1 Publication1

<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

O-glycosylated.By similarity

Keywords - PTMi

Glycoprotein, Lipoprotein, Oxidation, Phosphoprotein, Prenylation

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P09936

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

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PaxDbi
P09936

PeptideAtlas

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PeptideAtlasi
P09936

PRoteomics IDEntifications database

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PRIDEi
P09936

ProteomicsDB human proteome resource

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ProteomicsDBi
52282

Consortium for Top Down Proteomics

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TopDownProteomicsi
P09936

2D gel databases

DOSAC-COBS 2D-PAGE database

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DOSAC-COBS-2DPAGEi
P09936

University College Dublin 2-DE Proteome Database

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UCD-2DPAGEi
P09936

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P09936

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

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PhosphoSitePlusi
P09936

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
P09936

<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

Found in neuronal cell bodies and processes throughout the neocortex (at protein level). Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. Down-regulated in brains from Parkinson disease and Alzheimer disease patients.2 Publications

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000154277 Expressed in 199 organ(s), highest expression level in anterior cingulate cortex

CleanEx database of gene expression profiles

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CleanExi
HS_UCHL1

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P09936 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P09936 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
CAB002580
CAB079024
HPA005993

<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. Homodimer. Interacts with SNCA (By similarity). Interacts with COPS5.By similarity3 Publications

<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

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
113192, 61 interactors

CORUM comprehensive resource of mammalian protein complexes

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CORUMi
P09936

Database of interacting proteins

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DIPi
DIP-36620N

Protein interaction database and analysis system

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IntActi
P09936, 29 interactors

Molecular INTeraction database

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MINTi
P09936

STRING: functional protein association networks

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STRINGi
9606.ENSP00000284440

Chemistry databases

BindingDB database of measured binding affinities

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BindingDBi
P09936

<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

1223
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P09936

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

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SMRi
P09936

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
P09936

<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

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>Regioni5 – 10Interaction with ubiquitin1 Publication6
Regioni211 – 216Interaction with ubiquitin1 Publication6

<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 C12 family.Curated

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG1415 Eukaryota
ENOG4111HNA LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000157306

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000182400

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG075483

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P09936

KEGG Orthology (KO)

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KOi
K05611

Database for complete collections of gene phylogenies

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PhylomeDBi
P09936

TreeFam database of animal gene trees

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TreeFami
TF316166

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...
Gene3Di
3.40.532.10, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR038765 Papain_like_cys_pep_sf
IPR001578 Peptidase_C12_UCH
IPR036959 Peptidase_C12_UCH_sf
IPR030297 UCHL1

The PANTHER Classification System

More...
PANTHERi
PTHR10589 PTHR10589, 1 hit
PTHR10589:SF19 PTHR10589:SF19, 1 hit

Pfam protein domain database

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Pfami
View protein in Pfam
PF01088 Peptidase_C12, 1 hit

Protein Motif fingerprint database; a protein domain database

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PRINTSi
PR00707 UBCTHYDRLASE

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF54001 SSF54001, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00140 UCH_1, 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_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>Sequence (1+)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 has 1 described isoform and 5 potential isoforms that are computationally mapped.Show allAlign All

P09936-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MQLKPMEINP EMLNKVLSRL GVAGQWRFVD VLGLEEESLG SVPAPACALL
60 70 80 90 100
LLFPLTAQHE NFRKKQIEEL KGQEVSPKVY FMKQTIGNSC GTIGLIHAVA
110 120 130 140 150
NNQDKLGFED GSVLKQFLSE TEKMSPEDRA KCFEKNEAIQ AAHDAVAQEG
160 170 180 190 200
QCRVDDKVNF HFILFNNVDG HLYELDGRMP FPVNHGASSE DTLLKDAAKV
210 220
CREFTEREQG EVRFSAVALC KAA
Length:223
Mass (Da):24,824
Last modified:November 1, 1990 - 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:iC9E972AC4DA5DA8A
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
D6R956D6R956_HUMAN
Ubiquitin carboxyl-terminal hydrola...
UCHL1
241Annotation 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>
D6RE83D6RE83_HUMAN
Ubiquitin carboxyl-terminal hydrola...
UCHL1
207Annotation 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>
D6R974D6R974_HUMAN
Ubiquitin carboxyl-terminal hydrola...
UCHL1
156Annotation 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>
D6RF53D6RF53_HUMAN
Ubiquitin carboxyl-terminal hydrola...
UCHL1
58Annotation 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>
D6RJD9D6RJD9_HUMAN
Ubiquitin carboxyl-terminal hydrola...
UCHL1
69Annotation 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 CAA28443 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0708757E → A in SPG79; has decreased binding to ubiquitin and significantly decreased hydrolase activity compared to wild-type. 1 PublicationCorresponds to variant dbSNP:rs397515634EnsemblClinVar.1
Natural variantiVAR_01567718S → Y Polymorphism; may be associated with reduced risk for sporadic Parkinson disease; it confers protection from oxidative stress when expressed at physiological levels in neuroblastoma cells and primary cortical neurons; loss of dimerization ability; impaired ligase activity. 8 PublicationsCorresponds to variant dbSNP:rs5030732EnsemblClinVar.1
Natural variantiVAR_01567893I → M in PARK5; impaired enzymatic hydrolase activity; has about a 50% reduction in catalytic activity compared to wild-type protein. 4 PublicationsCorresponds to variant dbSNP:rs121917767EnsemblClinVar.1
Natural variantiVAR_078119178R → Q in SPG79; increased hydrolase activity; decreased protein abundance. 1 PublicationCorresponds to variant dbSNP:rs768996179EnsemblClinVar.1
Natural variantiVAR_078120216A → D in SPG79; decreased protein abundance. 1 PublicationCorresponds to variant dbSNP:rs1057519600Ensembl.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
AC095043 Genomic DNA Translation: AAY40923.1
CH471069 Genomic DNA Translation: EAW92983.1
BC000332 mRNA Translation: AAH00332.1
BC005117 mRNA Translation: AAH05117.1
BC006305 mRNA Translation: AAH06305.1
X17377 Genomic DNA Translation: CAA35249.1
X04741 mRNA Translation: CAA28443.1 Different initiation.
AH007277 Genomic DNA Translation: AAD09172.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS3462.1

Protein sequence database of the Protein Information Resource

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PIRi
A25856

NCBI Reference Sequences

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RefSeqi
NP_004172.2, NM_004181.4

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.518731

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000284440; ENSP00000284440; ENSG00000154277
ENST00000503431; ENSP00000422542; ENSG00000154277

Database of genes from NCBI RefSeq genomes

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GeneIDi
7345

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:7345

Keywords - Coding sequence diversityi

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

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Wikipedia

Ubiquitin carboxy-terminal hydrolase L1 entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC095043 Genomic DNA Translation: AAY40923.1
CH471069 Genomic DNA Translation: EAW92983.1
BC000332 mRNA Translation: AAH00332.1
BC005117 mRNA Translation: AAH05117.1
BC006305 mRNA Translation: AAH06305.1
X17377 Genomic DNA Translation: CAA35249.1
X04741 mRNA Translation: CAA28443.1 Different initiation.
AH007277 Genomic DNA Translation: AAD09172.1
CCDSiCCDS3462.1
PIRiA25856
RefSeqiNP_004172.2, NM_004181.4
UniGeneiHs.518731

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2ETLX-ray2.40A/B1-223[»]
2LENNMR-A1-223[»]
3IFWX-ray2.40A1-223[»]
3IRTX-ray2.80A/B1-223[»]
3KVFX-ray2.80A1-223[»]
3KW5X-ray2.83A1-223[»]
4DM9X-ray2.35A/B1-223[»]
4JKJX-ray2.15A/B1-223[»]
ProteinModelPortaliP09936
SMRiP09936
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113192, 61 interactors
CORUMiP09936
DIPiDIP-36620N
IntActiP09936, 29 interactors
MINTiP09936
STRINGi9606.ENSP00000284440

Chemistry databases

BindingDBiP09936
ChEMBLiCHEMBL6159
GuidetoPHARMACOLOGYi2426

Protein family/group databases

MEROPSiC12.001

PTM databases

iPTMnetiP09936
PhosphoSitePlusiP09936
SwissPalmiP09936

Polymorphism and mutation databases

BioMutaiUCHL1
DMDMi136681

2D gel databases

DOSAC-COBS-2DPAGEiP09936
UCD-2DPAGEiP09936

Proteomic databases

EPDiP09936
PaxDbiP09936
PeptideAtlasiP09936
PRIDEiP09936
ProteomicsDBi52282
TopDownProteomicsiP09936

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
7345
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000284440; ENSP00000284440; ENSG00000154277
ENST00000503431; ENSP00000422542; ENSG00000154277
GeneIDi7345
KEGGihsa:7345

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
7345
DisGeNETi7345
EuPathDBiHostDB:ENSG00000154277.12

GeneCards: human genes, protein and diseases

More...
GeneCardsi
UCHL1
HGNCiHGNC:12513 UCHL1
HPAiCAB002580
CAB079024
HPA005993
MalaCardsiUCHL1
MIMi191342 gene
613643 phenotype
615491 phenotype
neXtProtiNX_P09936
OpenTargetsiENSG00000154277
Orphaneti352654 Early-onset progressive neurodegeneration-blindness-ataxia-spasticity syndrome
2828 Young-onset Parkinson disease
PharmGKBiPA37160

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG1415 Eukaryota
ENOG4111HNA LUCA
GeneTreeiENSGT00940000157306
HOGENOMiHOG000182400
HOVERGENiHBG075483
InParanoidiP09936
KOiK05611
PhylomeDBiP09936
TreeFamiTF316166

Enzyme and pathway databases

BRENDAi3.4.19.12 2681
ReactomeiR-HSA-5689603 UCH proteinases
SABIO-RKiP09936
SIGNORiP09936

Miscellaneous databases

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

More...
ChiTaRSi
UCHL1 human
EvolutionaryTraceiP09936

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
Ubiquitin_carboxy-terminal_hydrolase_L1

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

More...
GenomeRNAii
7345

Protein Ontology

More...
PROi
PR:P09936

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000154277 Expressed in 199 organ(s), highest expression level in anterior cingulate cortex
CleanExiHS_UCHL1
ExpressionAtlasiP09936 baseline and differential
GenevisibleiP09936 HS

Family and domain databases

Gene3Di3.40.532.10, 1 hit
InterProiView protein in InterPro
IPR038765 Papain_like_cys_pep_sf
IPR001578 Peptidase_C12_UCH
IPR036959 Peptidase_C12_UCH_sf
IPR030297 UCHL1
PANTHERiPTHR10589 PTHR10589, 1 hit
PTHR10589:SF19 PTHR10589:SF19, 1 hit
PfamiView protein in Pfam
PF01088 Peptidase_C12, 1 hit
PRINTSiPR00707 UBCTHYDRLASE
SUPFAMiSSF54001 SSF54001, 1 hit
PROSITEiView protein in PROSITE
PS00140 UCH_1, 1 hit

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 nameiUCHL1_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: P09936
Secondary accession number(s): Q4W5K6, Q71UM0
<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: July 1, 1989
Last sequence update: November 1, 1990
Last modified: December 5, 2018
This is version 209 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, Complete proteome, Direct protein sequencing, 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 4
    Human chromosome 4: 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
  7. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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