BCKDHB Antibody from MYBIOSOURCE INC.

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Antigenic SpecificityBCKDHB
Clonepolyclonal
Host SpeciesRabbit
Reactive Specieshuman, mouse, rat
Isotypen/a
Formataffinity purified
Size0.1 mL, 0.2 mL
Concentrationn/a
ApplicationsWestern Blot (WB), Immunohistochemistry (IHC)
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DescriptionBranched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein 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 transcript variants with different 3' non-coding regions, but encoding the same isoform.
ImmunogenImmunogen: Recombinant protein of human BCKDHB
Other Names[E1B; dJ279A18.1]
Gene, Accession #[BCKDHB], Gene ID: 594, NCBI: NP_000047.1, UniProt: P21953
Catalog #MBS9129423
Price$275, $430
Order / More InfoBCKDHB Antibody from MYBIOSOURCE INC.
Product Specific Referencesn/a
MYBIOSOURCE INC.
MYBIOSOURCE INC.
MYBIOSOURCE INC.
P.O. Box 153308
San Diego CA 92195-3308
P: 1.858.633.0165
P: 1.888.MBS.0165 (1.888.627.0165) (US & Canada)
F: 1.858.633.0166

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