Q9Y5J6 (TI10B_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

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 names

Name:	TIMM10B
Synonyms:	FXC1, TIM9B, TIMM9B

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	103 AA.
Sequence status	Complete.
Protein existence	Evidence 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.

Ontologies

Keywords
Biological process	Protein transport Translocation Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Polymorphism
Ligand	Metal-binding Zinc
PTM	Disulfide bond
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	cell-matrix adhesion Traceable author statement PubMed 9731230. Source: ProtInc cellular protein metabolic process Traceable author statement. Source: Reactome protein targeting to mitochondrion Traceable author statement. Source: Reactome
Cellular_component	mitochondrial inner membrane Inferred from direct assay Ref.6. Source: BHF-UCL mitochondrial intermembrane space protein transporter complex Inferred from direct assay Ref.6. Source: BHF-UCL
Molecular_function	metal ion binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 103	103	Mitochondrial import inner membrane translocase subunit Tim10 B		PRO_0000193598
Regions
	Motif	28 – 52	25	Twin CX3C motif
Amino acid modifications
	Disulfide bond	28 ↔ 52		By similarity
	Disulfide bond	32 ↔ 48		By similarity
Natural variations
	Natural variant	66	1	A → S. Corresponds to variant rs60702727 [ dbSNP | Ensembl ].		VAR_061843
	Natural variant	90	1	G → S. Ref.4 Corresponds to variant rs17850713 [ dbSNP | Ensembl ].		VAR_025665

Sequences

	Sequence		Length	Mass (Da)
	Q9Y5J6 [UniParc]. Last modified November 1, 1999. Version 1. Checksum: B76CCC81668B1F31	FASTA	103	11,586
	10 20 30 40 50 60 MERQQQQQQQ LRNLRDFLLV YNRMTELCFQ RCVPSLHHRA LDAEEEACLH SCAGKLIHSN 70 80 90 100 HRLMAAYVQL MPALVQRRIA DYEAASAVPG VAAEQPGVSP SGS

References

	« Hide 'large scale' referencesShow '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.
IPI	IPI00001538.
RefSeq	NP_036324.1. NM_012192.3.
UniGene	Hs.54943.
3D structure databases
ProteinModelPortal	Q9Y5J6.
SMR	Q9Y5J6. Positions 20-64.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q9Y5J6. 3 interactions.
STRING	9606.ENSP00000254616.
Polymorphism databases
DMDM	12230190.
Proteomic databases
PaxDb	Q9Y5J6.
PeptideAtlas	Q9Y5J6.
PRIDE	Q9Y5J6.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000254616; ENSP00000254616; ENSG00000132286. ENST00000533379; ENSP00000436948; ENSG00000132286.
GeneID	26515.
KEGG	hsa:26515.
UCSC	uc001mdn.4. human.
Organism-specific databases
CTD	26515.
GeneCards	GC11P006460.
HGNC	HGNC:4022. TIMM10B.
HPA	HPA052265.
MIM	607388. gene.
neXtProt	NX_Q9Y5J6.
PharmGKB	PA28438.
GenAtlas	Search...
Phylogenomic databases
eggNOG	NOG286575.
HOGENOM	HOG000059523.
HOVERGEN	HBG054232.
InParanoid	Q9Y5J6.
OMA	RMADYEA.
OrthoDB	EOG4W9J5G.
PhylomeDB	Q9Y5J6.
Enzyme and pathway databases
Reactome	REACT_17015. Metabolism of proteins.
Gene expression databases
ArrayExpress	Q9Y5J6.
Bgee	Q9Y5J6.
CleanEx	HS_FXC1.
Genevestigator	Q9Y5J6.
GermOnline	ENSG00000132286. Homo sapiens.
Family and domain databases
Gene3D	1.10.287.810. 1 hit.
InterPro	IPR004217. Tim10/DDP_fam_Znf. [Graphical view]
Pfam	PF02953. zf-Tim10_DDP. 1 hit. [Graphical view]
SUPFAM	SSF144122. SSF144122. 1 hit.
ProtoNet	Search...
Other
ChiTaRS	FXC1. human.
GenomeRNAi	26515.
NextBio	48824.
SOURCE	Search...

Entry information

Entry name

TI10B_HUMAN

Accession

Primary (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:	May 1, 2013
This is version 104 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.