Recombinant Full Length Human BCS1L Protein, GST-tagged

• Cat.No.: BCS1L-1559HF
• Product Overview: Human BCS1L full-length ORF ( NP_004319.1, 1 a.a. - 419 a.a.) recombinant protein with GST-tag at N-terminal.

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. [provided by RefSeq
• Source: In Vitro Cell Free System
• Species: Human
• Tag: GST
• Form: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.
• Molecular Mass: 73.9 kDa
• Protein Length: 419 amino acids
• AA Sequence: MPLSDFILAL KDNPYFGAGF GLVGVGTALA LARKGVQLGL VAFRRHYMIT LEVPARDRSY AWLLSWLTRH STRTQHLSVE TSYLQHESGR ISTKFEFVPS PGNHFIWYRG KWIRVERSRE MQMIDLQTGT PWESVTFTAL GTDRKVFFNI LEEARELALQ QEEGKTVMYT AVGSEWRPFG YPRRRRPLNS VVLQQGLADR IVRDVQEFID NPKWYTDRGI PYRRGYLLYG PPGCGKSSFI TALAGELEHS ICLLSLTDSS LSDDRLNHLL SVAPQQSLVL LEDVDAAFLS RDLAVENPVK YQGLGRLTFS GLLNALDGVA STEARIVFMT TNHVDRLDPA LIRPGRVDLK EYVGYCSHWQ LTQMFQRFYP GQAPSLAENF AEHVLRATNQ ISPAQVQGYF MLYKNDPVGA IHNAESLRR
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

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