Anti-BCKDHB Rabbit Polyclonal Antibody
Product Information
Downloads & Supports
Target
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BCKDHB
Host
Storage
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Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Clonality
Clone Number
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-
Isotype
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IgG
Reactivity
Application
Application Details
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WB: 1/500-1/1000
Immunogen
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Recombinant fusion protein
Molecular Weight
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Calculated MW:43kDa
Formulation
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Liquid in PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Form
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Liquid
Gene ID
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594
Uniprot ID
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P21953
Background
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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.
Research Area
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Signal Transduction
Synonyms
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E1B; BCKDE1B; BCKDH E1-beta; BCKDHB