Rabbit polyclonal BCS1L antibody (A7647)

A7647

Recombinant protein of human BCS1L

WB, IHC

Polyclonal

IgG

BCS1L antibody was tube-contained.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Store at -20 Celsius degree. Avoid freeze / thaw cycles.

Human UniProt/Swiss-Prot:Q9Y276

Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex.

Belongs to the AAA ATPase family. BCS1 subfamily.

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. Five alternatively spliced transcripts encoding the same protein have been described.

Primary antibody