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Q9Y5J6

- T10B_HUMAN

UniProt

Q9Y5J6 - T10B_HUMAN

Protein

Mitochondrial import inner membrane translocase subunit Tim10 B

Gene

TIMM10B

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

    Component of the TIM22 complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. The TIM22 complex forms a twin-pore translocase that uses the membrane potential as the external driving force. In the TIM22 complex, it may act as a docking point for the soluble 70 kDa complex that guides the target proteins in transit through the aqueous mitochondrial intermembrane space.1 Publication

    GO - Molecular functioni

    1. metal ion binding Source: UniProtKB-KW

    GO - Biological processi

    1. cell-matrix adhesion Source: ProtInc
    2. cellular protein metabolic process Source: Reactome
    3. protein targeting to mitochondrion Source: Reactome

    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 Tim10 B
    Alternative name(s):
    Fracture callus protein 1
    FxC1
    Mitochondrial import inner membrane translocase subunit Tim9 B
    TIMM10B
    Short name:
    Tim10b
    Gene namesi
    Name:TIMM10B
    Synonyms:FXC1, TIM9B, TIMM9B
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 11

    Organism-specific databases

    HGNCiHGNC:4022. TIMM10B.

    Subcellular locationi

    Mitochondrion inner membrane 2 Publications; Peripheral membrane protein 2 Publications

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: BHF-UCL
    2. mitochondrial intermembrane space Source: BHF-UCL
    3. mitochondrial intermembrane space protein transporter complex Source: BHF-UCL

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA28438.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 103103Mitochondrial import inner membrane translocase subunit Tim10 BPRO_0000193598Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Disulfide bondi28 ↔ 52By similarity
    Disulfide bondi32 ↔ 48By similarity

    Keywords - PTMi

    Disulfide bond

    Proteomic databases

    MaxQBiQ9Y5J6.
    PaxDbiQ9Y5J6.
    PeptideAtlasiQ9Y5J6.
    PRIDEiQ9Y5J6.

    Expressioni

    Tissue specificityi

    Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.2 Publications

    Gene expression databases

    ArrayExpressiQ9Y5J6.
    BgeeiQ9Y5J6.
    CleanExiHS_FXC1.
    GenevestigatoriQ9Y5J6.

    Organism-specific databases

    HPAiHPA052265.

    Interactioni

    Subunit structurei

    Component of the TIM22 complex, whose core is composed of TIMM22, associated with peripheral protein TIMM10B/FXC1 and the 70 kDa heterohexamer. In most cases, the 70 kDa complex is composed of TIMM9 and TIMM10. Also forms a complex composed of TIMM9, TIMM10/TIM10A and TIMM10B/FXC1.

    Protein-protein interaction databases

    BioGridi117720. 3 interactions.
    IntActiQ9Y5J6. 3 interactions.
    STRINGi9606.ENSP00000254616.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9Y5J6.
    SMRiQ9Y5J6. Positions 12-70.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi28 – 5225Twin 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 TIMM10B/FXC1 from the cytoplasm into the mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across the mitochondrial outer membrane By similarity.By similarity

    Sequence similaritiesi

    Belongs to the small Tim family.Curated

    Phylogenomic databases

    eggNOGiNOG286575.
    HOGENOMiHOG000059523.
    HOVERGENiHBG054232.
    InParanoidiQ9Y5J6.
    KOiK17779.
    OMAiRMADYEA.
    OrthoDBiEOG7288V7.
    PhylomeDBiQ9Y5J6.
    TreeFamiTF106188.

    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.

    Q9Y5J6-1 [UniParc]FASTAAdd to Basket

    « Hide

    MERQQQQQQQ LRNLRDFLLV YNRMTELCFQ RCVPSLHHRA LDAEEEACLH    50
    SCAGKLIHSN HRLMAAYVQL MPALVQRRIA DYEAASAVPG VAAEQPGVSP 100
    SGS 103
    Length:103
    Mass (Da):11,586
    Last modified:November 1, 1999 - v1
    Checksum:iB76CCC81668B1F31
    GO

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti66 – 661A → S.
    Corresponds to variant rs60702727 [ dbSNP | Ensembl ].
    VAR_061843
    Natural varianti90 – 901G → S.1 Publication
    Corresponds to variant rs17850713 [ dbSNP | Ensembl ].
    VAR_025665

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF152355 mRNA. Translation: AAF15105.1.
    AF150105 mRNA. Translation: AAD40011.1.
    AF183415 mRNA. Translation: AAG09684.1.
    BC011014 mRNA. Translation: AAH11014.1.
    CCDSiCCDS7766.1.
    RefSeqiNP_036324.1. NM_012192.3.
    UniGeneiHs.54943.

    Genome annotation databases

    EnsembliENST00000254616; ENSP00000254616; ENSG00000132286.
    ENST00000533379; ENSP00000436948; ENSG00000132286.
    GeneIDi26515.
    KEGGihsa:26515.
    UCSCiuc001mdn.4. human.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF152355 mRNA. Translation: AAF15105.1 .
    AF150105 mRNA. Translation: AAD40011.1 .
    AF183415 mRNA. Translation: AAG09684.1 .
    BC011014 mRNA. Translation: AAH11014.1 .
    CCDSi CCDS7766.1.
    RefSeqi NP_036324.1. NM_012192.3.
    UniGenei Hs.54943.

    3D structure databases

    ProteinModelPortali Q9Y5J6.
    SMRi Q9Y5J6. Positions 12-70.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 117720. 3 interactions.
    IntActi Q9Y5J6. 3 interactions.
    STRINGi 9606.ENSP00000254616.

    Proteomic databases

    MaxQBi Q9Y5J6.
    PaxDbi Q9Y5J6.
    PeptideAtlasi Q9Y5J6.
    PRIDEi Q9Y5J6.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000254616 ; ENSP00000254616 ; ENSG00000132286 .
    ENST00000533379 ; ENSP00000436948 ; ENSG00000132286 .
    GeneIDi 26515.
    KEGGi hsa:26515.
    UCSCi uc001mdn.4. human.

    Organism-specific databases

    CTDi 26515.
    GeneCardsi GC11P006502.
    HGNCi HGNC:4022. TIMM10B.
    HPAi HPA052265.
    MIMi 607388. gene.
    neXtProti NX_Q9Y5J6.
    PharmGKBi PA28438.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG286575.
    HOGENOMi HOG000059523.
    HOVERGENi HBG054232.
    InParanoidi Q9Y5J6.
    KOi K17779.
    OMAi RMADYEA.
    OrthoDBi EOG7288V7.
    PhylomeDBi Q9Y5J6.
    TreeFami TF106188.

    Enzyme and pathway databases

    Reactomei REACT_118595. Mitochondrial protein import.

    Miscellaneous databases

    ChiTaRSi FXC1. human.
    GeneWikii FXC1.
    GenomeRNAii 26515.
    NextBioi 48824.
    PROi Q9Y5J6.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q9Y5J6.
    Bgeei Q9Y5J6.
    CleanExi HS_FXC1.
    Genevestigatori Q9Y5J6.

    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 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].
    2. "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], TISSUE SPECIFICITY.
    3. "A novel gene expressed in human pheochromocytoma."
      Peng Y., Li Y., Jia J., Xu S., Han Z., Fu G., Chen Z.
      Submitted (SEP-1999) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Pheochromocytoma.
    4. "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], VARIANT SER-90.
      Tissue: Bone marrow.
    5. "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: SUBCELLULAR LOCATION.
    6. "Organization and function of the small Tim complexes acting along the import pathway of metabolite carriers into mammalian mitochondria."
      Muehlenbein N., Hofmann S., Rothbauer U., Bauer M.F.
      J. Biol. Chem. 279:13540-13546(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, INTERACTION WITH TIMM9; TIMM10 AND TIMM22.
    7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiT10B_HUMAN
    AccessioniPrimary (citable) accession number: Q9Y5J6
    Secondary accession number(s): Q96FF3
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: January 11, 2001
    Last sequence update: November 1, 1999
    Last modified: October 1, 2014
    This is version 116 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 11
      Human chromosome 11: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3