BCKDHB Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	WB
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	The immunogen for Anti-BCKDHB antibody is: synthetic peptide directed towards the N-terminal region of Human BCKDHB. Synthetic peptide located within the following region: GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDN
Formulation	Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Purification	Affinity Purified
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Predicted Protein Size	23 kDa
Gene Name	branched chain keto acid dehydrogenase E1, beta polypeptide
Database Link	NP_000047 Entrez Gene 594 Human UniProt ID P21953
Background	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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Synonyms	BCKDE1B; BCKDH E1-beta; E1B
Note	Immunogen Sequence Homology: Human: 100%; Dog: 93%; Horse: 93%; Bovine: 93%; Rabbit: 93%; Pig: 86%; Rat: 79%
Reference Data
Protein Pathways	Metabolic pathways, Valine, leucine and isoleucine degradation

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