Recombinant Human BCS1L cell lysate

• Cat.No.: BCS1L-167HCL

Specification

• Description: 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.
• Species: Human
• Size: 100 ul
• Storage Buffer: 1X Sample Buffer (50 mM Tris-HCl, 2% SDS, 10% glycerol, 300 mM 2-mercaptoethanol, 0.01% Bromophenol blue)
• Applications: Western Blot

Gene Information

• Gene Name: BCS1L BCS1-like (S. cerevisiae) [ Homo sapiens ]
• Official Symbol: BCS1L
• Synonyms: BCS1L; BCS1-like (S. cerevisiae); BCS1 (yeast homolog) like , BCS1 like (yeast); mitochondrial chaperone BCS1; BCS; Bjornstad syndrome; BJS; GRACILE syndrome; h BCS; Hs.6719; h-BCS1; BCS1-like protein; mitochondrial complex III assembly; PTD; BCS1; FLNMS; h-BCS; GRACILE
• Gene ID: 617
• mRNA Refseq: NM_001079866
• Protein Refseq: NP_001073335
• MIM: 603647
• UniProt ID: Q9Y276
• Chromosome Location: 2q35
• Pathway: Metabolism of proteins, organism-specific biosystem; Mitochondrial Protein Import, organism-specific biosystem
• Function: ATP binding; nucleoside-triphosphatase activity; nucleotide binding; protein binding