BCKDHB (NM_000056) Human Recombinant Protein

CAT#: TP761638

Purified recombinant protein of Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform 1 precursor (BCKDHB), full length, with N-terminal GST and C-terminal HIS tag, expressed in E. coli, 50ug

Product Images

Specifications

Product Data

• Species: Human
• Expression Host: E. coli
• Expression cDNA Clone or AA Sequence:
  Protein Sequence

  A DNA sequence encoding human full-length BCKDHB
• Tag: N-GST and C-His
• Predicted MW: 65.8 kDa
• Concentration: >50 ug/mL as determined by microplate BCA method
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining
• Buffer: 25mM Tris, pH8.0,150mM NaCl,10% glycerol,1%Sarkosyl.
• Storage: Store at -80°C.
• Stability: Stable for 12 months from the date of receipt of the product under proper storage and handling conditions. Avoid repeated freeze-thaw cycles.

Reference Data

• RefSeq: NP_000047
• Locus ID: 594
• UniProt ID: P21953, A0A140VKB3
• Cytogenetics: 6q14.1
• Refseq Size: 1552
• Refseq ORF: 1176
• Synonyms: BCKDE1B; BCKDH E1-beta; E1B
• Summary: 'This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]'
• Protein Pathways: Metabolic pathways, Valine, leucine and isoleucine degradation