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Protein

Mitochondrial import inner membrane translocase subunit Tim10 B

Gene

TIMM10B

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

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
Complete GO annotation...

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
Complete GO annotation...

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

BgeeiQ9Y5J6.
CleanExiHS_FXC1.
ExpressionAtlasiQ9Y5J6. baseline and differential.
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. 5 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.
GeneTreeiENSGT00450000040326.
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

        10         20         30         40         50
MERQQQQQQQ LRNLRDFLLV YNRMTELCFQ RCVPSLHHRA LDAEEEACLH
60 70 80 90 100
SCAGKLIHSN HRLMAAYVQL MPALVQRRIA DYEAASAVPG VAAEQPGVSP

SGS
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:
EMBLi
GenBanki
DDBJi
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.
ENST00000472836; ENSP00000419704; ENSG00000265264.
ENST00000533379; ENSP00000436948; ENSG00000132286.
GeneIDi26515.
KEGGihsa:26515.
UCSCiuc001mdn.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

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

Protein-protein interaction databases

BioGridi117720. 5 interactions.
IntActiQ9Y5J6. 3 interactions.
STRINGi9606.ENSP00000254616.

Proteomic databases

MaxQBiQ9Y5J6.
PaxDbiQ9Y5J6.
PeptideAtlasiQ9Y5J6.
PRIDEiQ9Y5J6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000254616; ENSP00000254616; ENSG00000132286.
ENST00000472836; ENSP00000419704; ENSG00000265264.
ENST00000533379; ENSP00000436948; ENSG00000132286.
GeneIDi26515.
KEGGihsa:26515.
UCSCiuc001mdn.4. human.

Organism-specific databases

CTDi26515.
GeneCardsiGC11P006502.
HGNCiHGNC:4022. TIMM10B.
HPAiHPA052265.
MIMi607388. gene.
neXtProtiNX_Q9Y5J6.
PharmGKBiPA28438.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG286575.
GeneTreeiENSGT00450000040326.
HOGENOMiHOG000059523.
HOVERGENiHBG054232.
InParanoidiQ9Y5J6.
KOiK17779.
OMAiRMADYEA.
OrthoDBiEOG7288V7.
PhylomeDBiQ9Y5J6.
TreeFamiTF106188.

Enzyme and pathway databases

ReactomeiREACT_118595. Mitochondrial protein import.

Miscellaneous databases

GeneWikiiFXC1.
GenomeRNAii26515.
NextBioi48824.
PROiQ9Y5J6.
SOURCEiSearch...

Gene expression databases

BgeeiQ9Y5J6.
CleanExiHS_FXC1.
ExpressionAtlasiQ9Y5J6. baseline and differential.
GenevestigatoriQ9Y5J6.

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.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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: January 7, 2015
This is version 119 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.