Reviewed, UniProtKB/Swiss-Prot Q9Y5J6 (TIM9B_HUMAN)

Names and origin

Protein names

Recommended name:
    Mitochondrial import inner membrane translocase subunit Tim9 B
Alternative name(s):
    TIMM10B
      Short name=Tim10b
    Fracture callus protein 1
    FxC1

Gene names

Name:	FXC1
Synonyms:	TIM9B, TIMM9B

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	103 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
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 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.
Subunit structure	Component of the TIM22 complex, whose core is composed of TIMM22, associated with peripheral protein FXC1/TIM10B 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 FXC1/TIM10B.
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein.
Tissue specificity	Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.
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 FXC1/TIM10B from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across 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
Gene Ontology (GO)
Biological process	cell-matrix adhesion Traceable author statement. Source: ProtInc
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 Tim9 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	90	1	G → S: dbSNP rs17850713.		VAR_025665

Sequences

	Sequence		Length	Mass (Da)
	Q9Y5J6-1 [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: 10552927] [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: 10611480] [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: 15489334] [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: 11489896] [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: 14726512] [Abstract] Cited for: FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, INTERACTION WITH TIMM9; TIMM10 AND TIMM22.

Cross-references

Sequence databases
EMBLGenBankDDBJ	AF152355 mRNA. Translation: AAF15105.1. AF150105 mRNA. Translation: AAD40011.1. AF183415 mRNA. Translation: AAG09684.1. BC011014 mRNA. Translation: AAH11014.1.
RefSeq	NP_036324.1.
UniGene	Hs.54943
3D structure databases
ModBase	Search...
Proteomic databases
PeptideAtlas	Q9Y5J6.
Genome annotation databases
Ensembl	ENSG00000132286. Homo sapiens. [Contig view]
GeneID	26515.
KEGG	hsa:26515.
Organism-specific databases
H-InvDB	HIX0009401.
HGNC	HGNC:4022. FXC1.
MIM	607388. gene.
PharmGKB	PA28438.
GenAtlas	Search...
GeneCards	Search...
Phylogenomic databases
HOGENOM	Q9Y5J6.
HOVERGEN	Q9Y5J6.
Gene expression databases
ArrayExpress	Q9Y5J6.
CleanEx	HS_FXC1.
GermOnline	ENSG00000132286. Homo sapiens.
Family and domain databases
InterPro	IPR004217. Znf_Tim10_DDP. [Graphical view]
Pfam	PF02953. zf-Tim10_DDP. 1 hit. [Graphical view]
BLOCKS	Search...
ProtoNet	Search...
Other Resources
NextBio	48824.
SOURCE	Search...

Entry information

Entry name

TIM9B_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:	November 4, 2008
This is version 60 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)