BCS1L antibody (clone 5F3), anti-human

Background:
This gene 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.

Other Names:
Mitochondrial chaperone BCS1, h-BCS1, BCS1-like protein, BCS1L, BCS1

Antigen Type:
Recombinant Protein

Gene Name: BCS1L
Gene ID: 617
NCBI Accession: NP_001073335.1;NP_001244271.1;NP_001244272.1;NP_001244273.1;NP_004319.1
Primary Accession: Q9Y276
Other Accession: NM_004328
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .