CPLM-000884

Genbank Nucleotide ID

Mitochondrial import inner membrane translocase subunit Tim8 A

Protein Synonyms/Alias

Deafness dystonia protein 1; X-linked deafness dystonia protein

Homo sapiens (Human)

Position	Peptide	Type	References
86	NRLEQTQKSKPVFSE	ubiquitination	[1, 2, 3, 4, 5]
88	LEQTQKSKPVFSESL	ubiquitination	[1, 2, 3, 4, 6, 7]

[1] A data set of human endogenous protein ubiquitination sites.
Shi Y, Chan DW, Jung SY, Malovannaya A, Wang Y, Qin J.
Mol Cell Proteomics. 2011 May;10(5):M110.002089. [PMID: 20972266]
[2] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
[3] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
[4] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
Chen Z, Zhou Y, Song J, Zhang Z.
Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789]
[5] Integrated proteomic analysis of post-translational modifications by serial enrichment.
Mertins P, Qiao JW, Patel J, Udeshi ND, Clauser KR, Mani DR, Burgess MW, Gillette MA, Jaffe JD, Carr SA.
Nat Methods. 2013 Jul;10(7):634-7. [PMID: 23749302]
[6] Systematic and quantitative assessment of the ubiquitin-modified proteome.
Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
[7] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]

Functional Description

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. Probably necessary for normal neurologic development.

MOTIF 43 66 Twin CX3C motif.
MOD_RES 94 94 Phosphoserine.
MOD_RES 96 96 Phosphoserine.
DISULFID 43 66 By similarity.
DISULFID 47 62 By similarity.

Chaperone; Complete proteome; Deafness; Disease mutation; Disulfide bond; Membrane; Metal-binding; Mitochondrion; Mitochondrion inner membrane; Phosphoprotein; Protein transport; Reference proteome; Translocation; Transport; Zinc.

UniProt (SWISSPROT/TrEMBL); GenBank; EMBL

GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell.
GO:0005758; C:mitochondrial intermembrane space; IDA:BHF-UCL.
GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
GO:0044267; P:cellular protein metabolic process; TAS:Reactome.
GO:0072321; P:chaperone-mediated protein transport; TAS:BHF-UCL.
GO:0007399; P:nervous system development; TAS:ProtInc.
GO:0006626; P:protein targeting to mitochondrion; TAS:Reactome.

IPR004217; Tim10/DDP_fam_Znf.

PF02953; zf-Tim10_DDP