BCS1L Products

Description

BCS1L encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Two alternatively spliced transcripts encoding the same protein have been described. [provided by RefSeq]

Bioinformatics

Entrez	617 Human
Uniprot	NP_001073335 Human NP_004319 Human NP_004319.1 Human
Product By Gene ID	617
Alternate Names	BCS BCS1 (yeast homolog)-like BCS1 BCS1-like (S. cerevisiae) BCS1-like (yeast) BCS1-like protein BJSGRACILE FLNMS h-BCS h-BCS1 mitochondrial chaperone BCS1 mitochondrial complex III assembly PTD