DNAJC19
entrez
131118
canonical
uniprot
Q96DA6
canonical
ensembl
ENSG00000205981
resolver
ensp
ENSP00000372005
resolver
ensp
ENSP00000419191
resolver
genesymbol
DNAJC19
resolver
genesymbol_syn
TIM14
resolver
genesymbol_syn
TIMM14
resolver
hgnc
HGNC:30528
resolver
uniprot
A0A0S2Z5X1
resolver
uniprot
B2R4B1
resolver
uniprot
C9JBV1
resolver
uniprot_entry
A0A0S2Z5X1_HUMAN
resolver
uniprot_entry
TIM14_HUMAN
resolver
uniprot_sec
B2R4B1
resolver
uniprot_sec
C9JBV1
resolver
amino_acid_sequence
MASTVVAVGLTIAAAGFAGRYVLQAMKHMEPQVKQVFQSLPKSAFSGGYYRGGFEPKMTKREAALILGVSPTANKGKIRDAHRRIMLLNHPDKGGSPYIAAKINEAKDLLEGQAKK
uniprot
proteins
disease_involvement
DISEASE: 3-methylglutaconic aciduria 5 (MGCA5) [MIM:610198]: An autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid. {ECO:0000269|PubMed:16055927}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: Mitochondrial co-chaperone which forms a complex with prohibitins to regulate cardiolipin remodeling (By similarity). May be a component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity). {ECO:0000250|UniProtKB:Q07914, ECO:0000250|UniProtKB:Q9CQV7}.
uniprot
proteins
mass_dalton
12499
uniprot
proteins
protein_family
TIM14 family
uniprot
proteins
pubmed
12592411
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16055927
uniprot
proteins
pubmed
16641997
uniprot
proteins
pubmed
19413330
uniprot
proteins
pubmed
20068231
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
25944712
uniprot
proteins
sequence_length
116
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000305|PubMed:12592411}; Single-pass membrane protein {ECO:0000305|PubMed:12592411}; Matrix side {ECO:0000250|UniProtKB:Q9CQV7}.
uniprot
proteins
transmembrane_region
TRANSMEM 4..24; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins