Reviewed, UniProtKB/Swiss-Prot Q9Y5L4 (TIM13_HUMAN)

Names and origin

Protein names

Recommended name:
    Mitochondrial import inner membrane translocase subunit Tim13

Gene names

Name:	TIMM13
Synonyms:	TIM13B, TIMM13A, TIMM13B

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	95 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at protein level.

General annotation (Comments)

Function	Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins.
Subunit structure	Heterohexamer; composed of 3 copies of TIMM8 (TIMM8A or TIMM8B) and 3 copies of TIMM13, named soluble 70 kDa complex. Associates with the TIM22 complex, whose core is composed of TIMM22.
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.
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 TIMM13 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
Ligand	Metal-binding Zinc
Molecular function	Chaperone
Gene Ontology (GO)
Biological process	protein targeting to mitochondrion Ref.2 Traceable author statement. Source: ProtInc sensory perception of sound Ref.2 Traceable author statement. Source: ProtInc
Cellular component	mitochondrial inner membrane presequence translocase complex Ref.2 Traceable author statement. Source: ProtInc
Molecular function	protein binding Inferred from physical interaction. Source: IntAct zinc ion binding Ref.2 Traceable author statement. Source: ProtInc
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
CAB39	Q9Y376	1	EBI-1057344,EBI-306905
CUL2	Q13617	1	EBI-1057344,EBI-456179
MOBKL3	Q9Y3A3	1	EBI-1057344,EBI-713935
TIMM8A	O60220	1	EBI-1057344,EBI-1049822
TIMM8B	Q9Y5J9	1	EBI-1057344,EBI-1049085

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 95	95	Mitochondrial import inner membrane translocase subunit Tim13		PRO_0000193623
Regions
	Motif	46 – 69	24	Twin CX3C motif
Amino acid modifications
	Disulfide bond	46 ↔ 69		By similarity
	Disulfide bond	50 ↔ 65		By similarity
Experimental info
	Sequence conflict	2 – 3	2	EG → DS in AAF15101. Ref.2
	Sequence conflict	12 – 14	3	SGS → TGG in AAF15101. Ref.2
	Sequence conflict	21	1	L → A in AAF15101. Ref.2

Sequences

	Sequence		Length	Mass (Da)
	Q9Y5L4-1 [UniParc]. Last modified November 1, 1999. Version 1. Checksum: E40E742C7CA55834	FASTA	95	10,500
	10 20 30 40 50 60 MEGGFGSDFG GSGSGKLDPG LIMEQVKVQI AVANAQELLQ RMTDKCFRKC IGKPGGSLDN 70 80 90 SEQKCIAMCM DRYMDAWNTV SRAYNSRLQR ERANM

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"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].
[2]	"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].
[3]	"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]. Tissue: Placenta.
[4]	"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: FUNCTION, SUBCELLULAR LOCATION, ZINC-BINDING, INTERACTION WITH TIMM8A.
[5]	"The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex." Roesch K., Hynds P.J., Varga R., Tranebjaerg L., Koehler C.M. Hum. Mol. Genet. 13:2101-2111(2004) [PubMed: 15254020] [Abstract] Cited for: FUNCTION.

Cross-references

Sequence databases
EMBLGenBankDDBJ	AF144700 mRNA. Translation: AAD39951.1. AF152351 mRNA. Translation: AAF15101.1. AF152352 mRNA. Translation: AAF15102.1. BC008607 mRNA. Translation: AAH08607.1.
RefSeq	NP_036590.1.
UniGene	Hs.75056
3D structure databases
ModBase	Search...
Protein-protein interaction databases
IntAct	Q9Y5L4.
Proteomic databases
PeptideAtlas	Q9Y5L4.
Genome annotation databases
Ensembl	ENSG00000099800. Homo sapiens. [Contig view]
GeneID	26517.
KEGG	hsa:26517.
Organism-specific databases
H-InvDB	HIX0014617. HIX0066910.
HGNC	HGNC:11816. TIMM13.
MIM	607383. gene.
PharmGKB	PA36522.
GenAtlas	Search...
GeneCards	Search...
Phylogenomic databases
HOGENOM	Q9Y5L4.
HOVERGEN	Q9Y5L4.
Gene expression databases
ArrayExpress	Q9Y5L4.
CleanEx	HS_TIMM13.
GermOnline	ENSG00000099800. 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
LinkHub	Q9Y5L4.
NextBio	48830.
SOURCE	Search...

Entry information

Entry name

TIM13_HUMAN

Accession

Primary (citable) accession number: Q9Y5L4
Secondary accession number(s): P62206, Q9UHL8, Q9WTL1

Entry history

Integrated into UniProtKB/Swiss-Prot:	January 11, 2001
Last sequence update:	November 1, 1999
Last modified:	November 4, 2008
This is version 61 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)