Recombinant Full Length Human BCKDHB Protein, C-Flag-tagged

• Cat.No.: BCKDHB-1763HFL
• Product Overview: Recombinant Full Length Human BCKDHB Protein, fused to Flag-tag at C-terminus, was expressed in Mammalian cells.

Specification

• Description: 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.
• Source: Mammalian cells
• Species: Human
• Tag: Flag
• Form: 25 mM Tris HCl, pH 7.3, 100 mM glycine, 10% glycerol.
• Molecular Mass: 37.8 kDa
• AA Sequence: MAVVAAAAGWLLRLRAAGAEGHWRR LPGAGLARGFLHPAATVEDAAQRRQ VAHFTFQPDPEPREYGQTQK MNLFQSVTSALDNSLAKDPTAVIFG EDVAFGGVFRCTVGLRDKYGKDRVF NTPLCEQGIVGFGIGIAVTG ATAIAEIQFADYIFPAFDQIVNEAA KYRYRSGDLFNCGSLTIRSPWGCVG HGALYHSQSPEAFFAHCPGI KVVIPRSPFQAKGLLLSCIEDKNPC IFFEPKILYRAAAEEVPIEPYNIPL SQAEVIQEGSDVTLVAWGTQ VHVIREVASMAKEKLGVSCEVIDLR TIIPWDVDTICKSVIKTGRLLISHE APLTGGFASEISSTVQEECF LNLEAPISRVCGYDTPFPHIFEPFY IPDKWKCYDALRKMINY TRTRPLEQKLISEEDLAANDILDYK DDDDKV
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining.
• Stability: Stable for 12 months from the date of receipt of the product under proper storage and handling conditions. Avoid repeated freeze-thaw cycles.
• Storage: Store at -80 centigrade.
• Concentration: >50 ug/mL as determined by microplate BCA method.
• Preparation: Recombinant protein was captured through anti-DDK affinity column followed by conventional chromatography steps.
• Protein Pathways: Metabolic pathways, Valine, leucine and isoleucine degradation

Gene Information

• Gene Name: BCKDHB branched chain keto acid dehydrogenase E1 subunit beta [ Homo sapiens (human) ]
• Official Symbol: BCKDHB
• Synonyms: E1B; BCKDE1B; BCKDH E1-beta
• Gene ID: 594
• mRNA Refseq: NM_183050.4
• Protein Refseq: NP_898871.1
• MIM: 248611
• UniProt ID: P21953