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BCKDHB Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech
SDP

Catalog No. 501728934 Shop All Proteintech Group Inc Products
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50-172-8934 150 μL
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Catalog No. 50-172-8934 Supplier Proteintech Group Inc Supplier No. 136851AP150UL
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Rabbit Polyclonal Antibody

Branched-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.

Specifications

Antigen BCKDHB
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.19 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
Gene BCKDHB
Gene Accession No. P21953, P35738, Q6P3A8
Gene Alias BCKDHB, E1B
Gene Symbols BCKDHB
Host Species Rabbit
Immunogen BCKDHB Fusion Protein Ag4552
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 12040, 29711, 594
Target Species Human, Mouse, Rat
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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