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. Several alternatively spliced transcripts encoding two different isoforms have been described.
仕様
Synonyms：BCS 1,BCS 1L,BCS,BCS1 （yeast homolog） like,BCS1,BCS1 like （S. cerevisiae）,BCS1 like,BCS1 like protein,Bjornstad syndrome （pili torti and deafness）,BJS,FLNMS,GRACILE,h BCS,H BCS1,Hs.6719,Mitochondrial chaperone BCS1,Mitochondrial complex III assembly,PTD
Host：Rabbit
Reactivity：Human,Mouse,Rat
Applications：WB,IHC
Concentration：1mg/ml
Immunogen：Recombinant protein of human BCS1L
Purification Method：Affinity purification
Clonality：Polyclonal
Conjugation：Unconjugated
Buffer：Buffer： PBS with 0.02％ sodium azide, 50％ glycerol, pH7.3.
Dilution：WB 1：500 - 1：2000 IHC 1：50 - 1：200
Gene_ID（human）：617
Swissprot：Q9Y276
Isotype：IgG
Research Areas：Cancer, Metabolism, Neuroscience, Signal transduction
