Anti-BCKDHB Antibody
Boster Bio Anti-BCKDHB Antibody catalog # A05595-1. Tested in WB, IHC, ELISA. This antibody reacts with Human, Mouse, Rat.
Product Specifications
Background
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain (BCKDHB; BCKDE1B; BCKDH E1-beta) is a subunit of the BCKDH complex, which is a mitochondrial enzyme in the degradation pathway for branched-chain amino acids (BCAA) . Together with BCKDHA, BCKDHB forms the E1 subunit of this complex, whereas DBT and DLD are the E2 and E3 subunits, respectively. A deficiency of the BCKDH complex in humans causes maple syrup urine disease (MSUD), a severe neurometabolic disorder diagnosed by the detection alloisoleucine in plasma (MIM 248600) . (PMID: 30709776)
Gene Name
BCKDHB
UniProt
P21953
Host
Rabbit
Reactivity
Human, Mouse, Rat
Immunogen
E.coli-derived human BCKDHB recombinant protein (Position: E43-M389) .
Clonality
Polyclonal
Applications
WB, IHC, ELISA
Purification
Immunogen affinity purified.
Concentration
500 μg/mL
Form
Liquid
Storage Conditions
12 months from date of receipt, -20°C as supplied. 6 months 2 to 8°C after reconstitution. Avoid repeated freezing and thawing.
Contents
500 μg/mL antibody with PBS,0.02% NaN3, 1 mg BSA and 50% glycerol.
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