dbPAF Protein Information

Tag Content
dbPAF ID dbPAF-0002044
Uniprot Accession O60220; TIM8A_HUMAN; B2R5A6; Q6IRW6;
Genbank Protein ID NP_004076.1;
Genbank Nucleotide ID NM_004085.3;
Protein Name Mitochondrial import inner membrane translocase subunit Tim8 A
Protein Synonyms/Alias Deafness dystonia protein 1;X-linked deafness dystonia protein;
Gene Name TIMM8A
Gene Synonyms/Alias DDP;DDP1;TIM8A;
Organism Homo sapiens(Human)
NCBI Taxa ID 9606
Functional Description
(View all)		 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.
Phosphorylation Sites
 dbPAF PTMs: 5
PositionPeptidesSourceReferences ( PMIDs )
57KPGPKLDSRAEACFVPhosphoSitePlus;curated22135298; 20833797
87RLEQTQKSKPVFSESPHOSIDA;curated21081558; 23911959
92QKSKPVFSESLSD--PHOSIDA;curated21081558; 24961811
94SKPVFSESLSD----dbPTM 3.0;Phospho.ELM 9.0;HPRD 9;PhosphoSitePlus;SysPTM 2.0;UniProt;curated18669648; 16381945; 23193290; 18988627; 22135298; 19366988;
96PVFSESLSD------dbPTM 3.0;Phospho.ELM 9.0;HPRD 9;PHOSIDA;PhosphoSitePlus;UniProt;curated18669648; 16381945; 23193290; 21406692; 22115753; 19651622;
Sequence
(Fasta)		MDSSSSSSAA GLGAVDPQLQ HFIEVETQKQ RFQQLVHQMT ELCWEKCMDK PGPKLDSRAE 60
ACFVNCVERF IDTSQFILNR LEQTQKSKPV FSESLSD 98
Keyword

KW-0143--Chaperone
KW-0181--Complete proteome
KW-0209--Deafness
KW-0225--Disease mutation
KW-1015--Disulfide bond
KW-0472--Membrane
KW-0479--Metal-binding
KW-0496--Mitochondrion
KW-0999--Mitochondrion inner membrane
KW-0597--Phosphoprotein
KW-0653--Protein transport
KW-1185--Reference proteome
KW-0811--Translocation
KW-0813--Transport
KW-0862--Zinc
Interpro

IPR004217--Tim10/DDP_fam_Znf
PROSITE
Pfam

PF02953--zf-Tim10_DDP
Gene Ontology

GO:0005743--C:mitochondrial inner membrane
GO:0005758--C:mitochondrial intermembrane space
GO:0005739--C:mitochondrion
GO:0046872--F:metal ion binding
GO:0044267--P:cellular protein metabolic process
GO:0072321--P:chaperone-mediated protein transport
GO:0007399--P:nervous system development
GO:0006626--P:protein targeting to mitochondrion