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Q9Y5J6 (T10B_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 114. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended 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 names
Name:TIMM10B
Synonyms:FXC1, TIM9B, TIMM9B
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length103 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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. Ref.6

Subunit structure

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.

Subcellular location

Mitochondrion inner membrane; Peripheral membrane protein Ref.5 Ref.6.

Tissue specificity

Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle. Ref.2 Ref.6

Domain

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.

Sequence similarities

Belongs to the small Tim family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 103103Mitochondrial import inner membrane translocase subunit Tim10 B
PRO_0000193598

Regions

Motif28 – 5225Twin CX3C motif

Amino acid modifications

Disulfide bond28 ↔ 52 By similarity
Disulfide bond32 ↔ 48 By similarity

Natural variations

Natural variant661A → S.
Corresponds to variant rs60702727 [ dbSNP | Ensembl ].
VAR_061843
Natural variant901G → S. Ref.4
Corresponds to variant rs17850713 [ dbSNP | Ensembl ].
VAR_025665

Sequences

Sequence LengthMass (Da)Tools
Q9Y5J6 [UniParc].

Last modified November 1, 1999. Version 1.
Checksum: B76CCC81668B1F31

FASTA10311,586
        10         20         30         40         50         60 
MERQQQQQQQ LRNLRDFLLV YNRMTELCFQ RCVPSLHHRA LDAEEEACLH SCAGKLIHSN 

        70         80         90        100 
HRLMAAYVQL MPALVQRRIA DYEAASAVPG VAAEQPGVSP SGS 

« Hide

References

« Hide 'large scale' references
[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]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF152355 mRNA. Translation: AAF15105.1.
AF150105 mRNA. Translation: AAD40011.1.
AF183415 mRNA. Translation: AAG09684.1.
BC011014 mRNA. Translation: AAH11014.1.
CCDSCCDS7766.1.
RefSeqNP_036324.1. NM_012192.3.
UniGeneHs.54943.

3D structure databases

ProteinModelPortalQ9Y5J6.
SMRQ9Y5J6. Positions 12-70.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid117720. 3 interactions.
IntActQ9Y5J6. 3 interactions.
STRING9606.ENSP00000254616.

Proteomic databases

MaxQBQ9Y5J6.
PaxDbQ9Y5J6.
PeptideAtlasQ9Y5J6.
PRIDEQ9Y5J6.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000254616; ENSP00000254616; ENSG00000132286.
ENST00000533379; ENSP00000436948; ENSG00000132286.
GeneID26515.
KEGGhsa:26515.
UCSCuc001mdn.4. human.

Organism-specific databases

CTD26515.
GeneCardsGC11P006502.
HGNCHGNC:4022. TIMM10B.
HPAHPA052265.
MIM607388. gene.
neXtProtNX_Q9Y5J6.
PharmGKBPA28438.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG286575.
HOGENOMHOG000059523.
HOVERGENHBG054232.
InParanoidQ9Y5J6.
KOK17779.
OMARMADYEA.
OrthoDBEOG7288V7.
PhylomeDBQ9Y5J6.
TreeFamTF106188.

Enzyme and pathway databases

ReactomeREACT_17015. Metabolism of proteins.

Gene expression databases

ArrayExpressQ9Y5J6.
BgeeQ9Y5J6.
CleanExHS_FXC1.
GenevestigatorQ9Y5J6.

Family and domain databases

Gene3D1.10.287.810. 1 hit.
InterProIPR004217. Tim10/DDP_fam_Znf.
[Graphical view]
PfamPF02953. zf-Tim10_DDP. 1 hit.
[Graphical view]
SUPFAMSSF144122. SSF144122. 1 hit.
ProtoNetSearch...

Other

ChiTaRSFXC1. human.
GeneWikiFXC1.
GenomeRNAi26515.
NextBio48824.
PROQ9Y5J6.
SOURCESearch...

Entry information

Entry nameT10B_HUMAN
AccessionPrimary (citable) accession number: Q9Y5J6
Secondary accession number(s): Q96FF3
Entry history
Integrated into UniProtKB/Swiss-Prot: January 11, 2001
Last sequence update: November 1, 1999
Last modified: July 9, 2014
This is version 114 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 11

Human chromosome 11: entries, gene names and cross-references to MIM