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Q9Y5L4 (TIM13_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 120. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | 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 Tim13
Gene names
Name:TIMM13
Synonyms:TIM13B, TIMM13A, TIMM13B
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length95 AA.
Sequence statusComplete.
Protein existenceEvidence 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. Ref.4 Ref.5

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 Ref.4.

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.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 9595Mitochondrial import inner membrane translocase subunit Tim13
PRO_0000193623

Regions

Motif46 – 6924Twin CX3C motif

Amino acid modifications

Modified residue11N-acetylmethionine Ref.6
Modified residue531N6-succinyllysine By similarity
Disulfide bond46 ↔ 69 By similarity
Disulfide bond50 ↔ 65 By similarity

Experimental info

Sequence conflict2 – 32EG → DS in AAF15101. Ref.2
Sequence conflict12 – 143SGS → TGG in AAF15101. Ref.2
Sequence conflict211L → A in AAF15101. Ref.2

Sequences

Sequence LengthMass (Da)Tools
Q9Y5L4 [UniParc].

Last modified November 1, 1999. Version 1.
Checksum: E40E742C7CA55834

FASTA9510,500
        10         20         30         40         50         60 
MEGGFGSDFG GSGSGKLDPG LIMEQVKVQI AVANAQELLQ RMTDKCFRKC IGKPGGSLDN 

        70         80         90 
SEQKCIAMCM DRYMDAWNTV SRAYNSRLQR ERANM 

« Hide

References

« Hide '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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [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] [Europe PMC] [Abstract]
Cited for: FUNCTION.
[6]"Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.
Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT MET-1, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[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
AF144700 mRNA. Translation: AAD39951.1.
AF152351 mRNA. Translation: AAF15101.1.
AF152352 mRNA. Translation: AAF15102.1.
BC008607 mRNA. Translation: AAH08607.1.
CCDSCCDS12089.1.
RefSeqNP_036590.1. NM_012458.3.
UniGeneHs.75056.

3D structure databases

ProteinModelPortalQ9Y5L4.
SMRQ9Y5L4. Positions 35-89.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid117722. 28 interactions.
IntActQ9Y5L4. 7 interactions.
MINTMINT-3087260.
STRING9606.ENSP00000215570.

PTM databases

PhosphoSiteQ9Y5L4.

Proteomic databases

MaxQBQ9Y5L4.
PaxDbQ9Y5L4.
PeptideAtlasQ9Y5L4.
PRIDEQ9Y5L4.

Protocols and materials databases

DNASU26517.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000215570; ENSP00000215570; ENSG00000099800.
GeneID26517.
KEGGhsa:26517.
UCSCuc002lvx.1. human.

Organism-specific databases

CTD26517.
GeneCardsGC19M002425.
HGNCHGNC:11816. TIMM13.
HPAHPA048985.
MIM607383. gene.
neXtProtNX_Q9Y5L4.
PharmGKBPA36522.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG246901.
HOGENOMHOG000115759.
HOVERGENHBG079645.
InParanoidQ9Y5L4.
KOK17781.
OMAGSEETCL.
OrthoDBEOG7Q8CQP.
PhylomeDBQ9Y5L4.
TreeFamTF106194.

Enzyme and pathway databases

ReactomeREACT_17015. Metabolism of proteins.

Gene expression databases

BgeeQ9Y5L4.
CleanExHS_TIMM13.
GenevestigatorQ9Y5L4.

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

GeneWikiTIMM13.
GenomeRNAi26517.
NextBio48830.
PROQ9Y5L4.
SOURCESearch...

Entry information

Entry nameTIM13_HUMAN
AccessionPrimary (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: July 9, 2014
This is version 120 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 chromosome 19

Human chromosome 19: entries, gene names and cross-references to MIM