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Protein

Ubiquitin carboxyl-terminal hydrolase isozyme L1

Gene

Uchl1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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.1 Publication

Miscellaneous

In contrast to UCHL3, does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8.By similarity

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).

Kineticsi

  1. KM=116 nM for Ub-AMC1 Publication

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Active sitei90NucleophileCurated1
    Active sitei161Proton donorPROSITE-ProRule annotation1
    Sitei176Important for enzyme activityBy similarity1

    GO - Molecular functioni

    GO - Biological processi

    Keywordsi

    Molecular functionHydrolase, Ligase, Protease, Thiol protease
    Biological processUbl conjugation pathway

    Enzyme and pathway databases

    BRENDAi3.4.19.12 3474
    ReactomeiR-MMU-5689603 UCH proteinases

    Protein family/group databases

    MEROPSiC12.001

    Names & Taxonomyi

    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
    Gene namesi
    Name:Uchl1
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
    Proteomesi
    • UP000000589 Componenti: Chromosome 5

    Organism-specific databases

    MGIiMGI:103149 Uchl1

    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

    Pathology & Biotechi

    Disruption phenotypei

    Mice show sensory ataxia at an early stage, followed by motor ataxia at a later stage. They have reduced levels of monoubiquitin in the nervous system, and increased resistance to retinal ischemia.2 Publications

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Mutagenesisi30D → K: Abolishes enzymatic activity and ubiquitin binding. 1 Publication1
    Mutagenesisi90C → S: Abolishes enzymatic activity, but does not affect ubiquitin binding. 1 Publication1

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_00002110581 – 220Ubiquitin carboxyl-terminal hydrolase isozyme L1Add BLAST220
    PropeptideiPRO_0000414313221 – 223Removed in mature formBy similarity3

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei125PhosphoserineBy similarity1
    Lipidationi220S-farnesyl cysteineBy similarity1

    Post-translational modificationi

    O-glycosylated.By similarity

    Keywords - PTMi

    Glycoprotein, Lipoprotein, Phosphoprotein, Prenylation

    Proteomic databases

    EPDiQ9R0P9
    MaxQBiQ9R0P9
    PaxDbiQ9R0P9
    PeptideAtlasiQ9R0P9
    PRIDEiQ9R0P9

    2D gel databases

    REPRODUCTION-2DPAGEiIPI00313962
    Q9R0P9
    UCD-2DPAGEiQ9R0P9

    PTM databases

    iPTMnetiQ9R0P9
    PhosphoSitePlusiQ9R0P9
    SwissPalmiQ9R0P9

    Expressioni

    Tissue specificityi

    Expressed in brain, where it is found in neurons but not in oligodendrocytes or astrocytes. Found in the ganglion cell layer and the inner nuclear layer of the retina (at protein level). Expressed in brain and testis. In the brain, expression is at its lowest in replaceable neurons of hippocampus and olfactory bulb. Highly expressed in senescent pituitary.5 Publications

    Gene expression databases

    BgeeiENSMUSG00000029223 Expressed in 286 organ(s), highest expression level in dorsal root ganglion
    CleanExiMM_UCHL1
    GenevisibleiQ9R0P9 MM

    Interactioni

    Subunit structurei

    Monomer. Homodimer. Interacts with COPS5 and SNCA (By similarity).By similarity

    GO - Molecular functioni

    Protein-protein interaction databases

    BioGridi204423, 11 interactors
    IntActiQ9R0P9, 6 interactors
    MINTiQ9R0P9
    STRINGi10090.ENSMUSP00000031131

    Chemistry databases

    BindingDBiQ9R0P9

    Structurei

    3D structure databases

    ProteinModelPortaliQ9R0P9
    SMRiQ9R0P9
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Regioni5 – 10Interaction with ubiquitinBy similarity6
    Regioni211 – 216Interaction with ubiquitinBy similarity6

    Sequence similaritiesi

    Belongs to the peptidase C12 family.Curated

    Phylogenomic databases

    eggNOGiKOG1415 Eukaryota
    ENOG4111HNA LUCA
    GeneTreeiENSGT00510000046640
    HOVERGENiHBG075483
    InParanoidiQ9R0P9
    KOiK05611
    OMAiFISFVKG
    OrthoDBiEOG091G0LBS
    PhylomeDBiQ9R0P9
    TreeFamiTF316166

    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

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    Q9R0P9-1 [UniParc]FASTAAdd to basket
    « Hide
            10         20         30         40         50
    MQLKPMEINP EMLNKVLAKL GVAGQWRFAD VLGLEEETLG SVPSPACALL
    60 70 80 90 100
    LLFPLTAQHE NFRKKQIEEL KGQEVSPKVY FMKQTIGNSC GTIGLIHAVA
    110 120 130 140 150
    NNQDKLEFED GSVLKQFLSE TEKLSPEDRA KCFEKNEAIQ AAHDSVAQEG
    160 170 180 190 200
    QCRVDDKVNF HFILFNNVDG HLYELDGRMP FPVNHGASSE DSLLQDAAKV
    210 220
    CREFTEREQG EVRFSAVALC KAA
    Length:223
    Mass (Da):24,838
    Last modified:May 1, 2000 - v1
    Checksum:iF1402BF7B0C077EA
    GO

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Sequence conflicti149E → K in AAD51029 (PubMed:12559414).Curated1

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AB025313 mRNA Translation: BAA84083.1
    AF172334 mRNA Translation: AAD51029.1
    AK013729 mRNA Translation: BAB28976.1
    BC039177 mRNA Translation: AAH39177.1
    CCDSiCCDS19315.1
    RefSeqiNP_035800.2, NM_011670.2
    UniGeneiMm.29807

    Genome annotation databases

    EnsembliENSMUST00000031131; ENSMUSP00000031131; ENSMUSG00000029223
    GeneIDi22223
    KEGGimmu:22223
    UCSCiuc008xpf.2 mouse

    Similar proteinsi

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AB025313 mRNA Translation: BAA84083.1
    AF172334 mRNA Translation: AAD51029.1
    AK013729 mRNA Translation: BAB28976.1
    BC039177 mRNA Translation: AAH39177.1
    CCDSiCCDS19315.1
    RefSeqiNP_035800.2, NM_011670.2
    UniGeneiMm.29807

    3D structure databases

    ProteinModelPortaliQ9R0P9
    SMRiQ9R0P9
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi204423, 11 interactors
    IntActiQ9R0P9, 6 interactors
    MINTiQ9R0P9
    STRINGi10090.ENSMUSP00000031131

    Chemistry databases

    BindingDBiQ9R0P9

    Protein family/group databases

    MEROPSiC12.001

    PTM databases

    iPTMnetiQ9R0P9
    PhosphoSitePlusiQ9R0P9
    SwissPalmiQ9R0P9

    2D gel databases

    REPRODUCTION-2DPAGEiIPI00313962
    Q9R0P9
    UCD-2DPAGEiQ9R0P9

    Proteomic databases

    EPDiQ9R0P9
    MaxQBiQ9R0P9
    PaxDbiQ9R0P9
    PeptideAtlasiQ9R0P9
    PRIDEiQ9R0P9

    Protocols and materials databases

    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENSMUST00000031131; ENSMUSP00000031131; ENSMUSG00000029223
    GeneIDi22223
    KEGGimmu:22223
    UCSCiuc008xpf.2 mouse

    Organism-specific databases

    CTDi7345
    MGIiMGI:103149 Uchl1

    Phylogenomic databases

    eggNOGiKOG1415 Eukaryota
    ENOG4111HNA LUCA
    GeneTreeiENSGT00510000046640
    HOVERGENiHBG075483
    InParanoidiQ9R0P9
    KOiK05611
    OMAiFISFVKG
    OrthoDBiEOG091G0LBS
    PhylomeDBiQ9R0P9
    TreeFamiTF316166

    Enzyme and pathway databases

    BRENDAi3.4.19.12 3474
    ReactomeiR-MMU-5689603 UCH proteinases

    Miscellaneous databases

    ChiTaRSiUchl1 mouse
    PROiPR:Q9R0P9
    SOURCEiSearch...

    Gene expression databases

    BgeeiENSMUSG00000029223 Expressed in 286 organ(s), highest expression level in dorsal root ganglion
    CleanExiMM_UCHL1
    GenevisibleiQ9R0P9 MM

    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
    ProtoNetiSearch...

    Entry informationi

    Entry nameiUCHL1_MOUSE
    AccessioniPrimary (citable) accession number: Q9R0P9
    Secondary accession number(s): Q9R122
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 26, 2001
    Last sequence update: May 1, 2000
    Last modified: November 7, 2018
    This is version 158 of the entry and version 1 of the sequence. See complete history.
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families
    2. Peptidase families
      Classification of peptidase families and list of entries
    3. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
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    Main funding by: National Institutes of Health

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