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Q9Y5L4

- TIM13_HUMAN

UniProt

Q9Y5L4 - TIM13_HUMAN

Protein

Mitochondrial import inner membrane translocase subunit Tim13

Gene

TIMM13

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
  1. Functioni

    Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins.2 Publications

    GO - Molecular functioni

    1. zinc ion binding Source: ProtInc

    GO - Biological processi

    1. cellular protein metabolic process Source: Reactome
    2. chaperone-mediated protein transport Source: BHF-UCL
    3. protein targeting to mitochondrion Source: Reactome
    4. sensory perception of sound Source: ProtInc

    Keywords - Molecular functioni

    Chaperone

    Keywords - Biological processi

    Protein transport, Translocation, Transport

    Keywords - Ligandi

    Metal-binding, Zinc

    Enzyme and pathway databases

    ReactomeiREACT_118595. Mitochondrial protein import.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Mitochondrial import inner membrane translocase subunit Tim13
    Gene namesi
    Name:TIMM13
    Synonyms:TIM13B, TIMM13A, TIMM13B
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 19

    Organism-specific databases

    HGNCiHGNC:11816. TIMM13.

    Subcellular locationi

    Mitochondrion inner membrane 1 Publication; Peripheral membrane protein 1 Publication; Intermembrane side 1 Publication

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: UniProtKB-SubCell
    2. mitochondrial intermembrane space protein transporter complex Source: BHF-UCL
    3. mitochondrion Source: HPA
    4. nucleolus Source: HPA

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA36522.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 9595Mitochondrial import inner membrane translocase subunit Tim13PRO_0000193623Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei1 – 11N-acetylmethionine1 Publication
    Disulfide bondi46 ↔ 69By similarity
    Disulfide bondi50 ↔ 65By similarity
    Modified residuei53 – 531N6-succinyllysineBy similarity

    Keywords - PTMi

    Acetylation, Disulfide bond

    Proteomic databases

    MaxQBiQ9Y5L4.
    PaxDbiQ9Y5L4.
    PeptideAtlasiQ9Y5L4.
    PRIDEiQ9Y5L4.

    PTM databases

    PhosphoSiteiQ9Y5L4.

    Expressioni

    Tissue specificityi

    Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.

    Gene expression databases

    BgeeiQ9Y5L4.
    CleanExiHS_TIMM13.
    GenevestigatoriQ9Y5L4.

    Organism-specific databases

    HPAiHPA048985.

    Interactioni

    Subunit structurei

    Heterohexamer; composed of 3 copies of TIMM8 (TIMM8A or TIMM8B) and 3 copies of TIMM13, named soluble 70 kDa complex. Associates with the TIM22 complex, whose core is composed of TIMM22.

    Protein-protein interaction databases

    BioGridi117722. 29 interactions.
    IntActiQ9Y5L4. 8 interactions.
    MINTiMINT-3087260.
    STRINGi9606.ENSP00000215570.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9Y5L4.
    SMRiQ9Y5L4. Positions 35-89.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi46 – 6924Twin CX3C motifAdd
    BLAST

    Domaini

    The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM13 from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane By similarity.By similarity

    Sequence similaritiesi

    Belongs to the small Tim family.Curated

    Phylogenomic databases

    eggNOGiNOG246901.
    HOGENOMiHOG000115759.
    HOVERGENiHBG079645.
    InParanoidiQ9Y5L4.
    KOiK17781.
    OMAiGSEETCL.
    OrthoDBiEOG7Q8CQP.
    PhylomeDBiQ9Y5L4.
    TreeFamiTF106194.

    Family and domain databases

    Gene3Di1.10.287.810. 1 hit.
    InterProiIPR004217. Tim10/DDP_fam_Znf.
    [Graphical view]
    PfamiPF02953. zf-Tim10_DDP. 1 hit.
    [Graphical view]
    SUPFAMiSSF144122. SSF144122. 1 hit.

    Sequencei

    Sequence statusi: Complete.

    Q9Y5L4-1 [UniParc]FASTAAdd to Basket

    « Hide

    MEGGFGSDFG GSGSGKLDPG LIMEQVKVQI AVANAQELLQ RMTDKCFRKC   50
    IGKPGGSLDN SEQKCIAMCM DRYMDAWNTV SRAYNSRLQR ERANM 95
    Length:95
    Mass (Da):10,500
    Last modified:November 1, 1999 - v1
    Checksum:iE40E742C7CA55834
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti2 – 32EG → DS in AAF15101. (PubMed:10552927)Curated
    Sequence conflicti12 – 143SGS → TGG in AAF15101. (PubMed:10552927)Curated
    Sequence conflicti21 – 211L → A in AAF15101. (PubMed:10552927)Curated

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF144700 mRNA. Translation: AAD39951.1.
    AF152351 mRNA. Translation: AAF15101.1.
    AF152352 mRNA. Translation: AAF15102.1.
    BC008607 mRNA. Translation: AAH08607.1.
    CCDSiCCDS12089.1.
    RefSeqiNP_036590.1. NM_012458.3.
    UniGeneiHs.75056.

    Genome annotation databases

    EnsembliENST00000215570; ENSP00000215570; ENSG00000099800.
    GeneIDi26517.
    KEGGihsa:26517.
    UCSCiuc002lvx.1. human.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF144700 mRNA. Translation: AAD39951.1 .
    AF152351 mRNA. Translation: AAF15101.1 .
    AF152352 mRNA. Translation: AAF15102.1 .
    BC008607 mRNA. Translation: AAH08607.1 .
    CCDSi CCDS12089.1.
    RefSeqi NP_036590.1. NM_012458.3.
    UniGenei Hs.75056.

    3D structure databases

    ProteinModelPortali Q9Y5L4.
    SMRi Q9Y5L4. Positions 35-89.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 117722. 29 interactions.
    IntActi Q9Y5L4. 8 interactions.
    MINTi MINT-3087260.
    STRINGi 9606.ENSP00000215570.

    PTM databases

    PhosphoSitei Q9Y5L4.

    Proteomic databases

    MaxQBi Q9Y5L4.
    PaxDbi Q9Y5L4.
    PeptideAtlasi Q9Y5L4.
    PRIDEi Q9Y5L4.

    Protocols and materials databases

    DNASUi 26517.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000215570 ; ENSP00000215570 ; ENSG00000099800 .
    GeneIDi 26517.
    KEGGi hsa:26517.
    UCSCi uc002lvx.1. human.

    Organism-specific databases

    CTDi 26517.
    GeneCardsi GC19M002425.
    HGNCi HGNC:11816. TIMM13.
    HPAi HPA048985.
    MIMi 607383. gene.
    neXtProti NX_Q9Y5L4.
    PharmGKBi PA36522.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG246901.
    HOGENOMi HOG000115759.
    HOVERGENi HBG079645.
    InParanoidi Q9Y5L4.
    KOi K17781.
    OMAi GSEETCL.
    OrthoDBi EOG7Q8CQP.
    PhylomeDBi Q9Y5L4.
    TreeFami TF106194.

    Enzyme and pathway databases

    Reactomei REACT_118595. Mitochondrial protein import.

    Miscellaneous databases

    GeneWikii TIMM13.
    GenomeRNAii 26517.
    NextBioi 48830.
    PROi Q9Y5L4.
    SOURCEi Search...

    Gene expression databases

    Bgeei Q9Y5L4.
    CleanExi HS_TIMM13.
    Genevestigatori Q9Y5L4.

    Family and domain databases

    Gene3Di 1.10.287.810. 1 hit.
    InterProi IPR004217. Tim10/DDP_fam_Znf.
    [Graphical view ]
    Pfami PF02953. zf-Tim10_DDP. 1 hit.
    [Graphical view ]
    SUPFAMi SSF144122. SSF144122. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom."
      Bauer M.F., Rothbauer U., Muehlenbein N., Smith R.J.H., Gerbitz K.-D., Neupert W., Brunner M., Hofmann S.
      FEBS Lett. 464:41-47(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    2. "The human family of deafness/dystonia peptide (DDP) related mitochondrial import proteins."
      Jin H., Kendall E., Freeman T.C., Roberts R.G., Vetrie D.L.P.
      Genomics 61:259-267(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Placenta.
    4. "Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria."
      Rothbauer U., Hofmann S., Muehlenbein N., Paschen S.A., Gerbitz K.-D., Neupert W., Brunner M., Bauer M.F.
      J. Biol. Chem. 276:37327-37334(2001) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, SUBCELLULAR LOCATION, ZINC-BINDING, INTERACTION WITH TIMM8A.
    5. "The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex."
      Roesch K., Hynds P.J., Varga R., Tranebjaerg L., Koehler C.M.
      Hum. Mol. Genet. 13:2101-2111(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION.
    6. "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
      Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.
      Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT MET-1, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiTIM13_HUMAN
    AccessioniPrimary (citable) accession number: Q9Y5L4
    Secondary accession number(s): P62206, Q9UHL8, Q9WTL1
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: January 11, 2001
    Last sequence update: November 1, 1999
    Last modified: October 1, 2014
    This is version 122 of the entry and version 1 of the sequence. [Complete history]
    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.

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 19
      Human chromosome 19: entries, gene names and cross-references to MIM
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3