BCKDHA Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IHC, WB
Recommend Dilution	WB
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	The immunogen for anti-BCKDHA antibody: synthetic peptide directed towards the N terminal of human BCKDHA. Synthetic peptide located within the following region: NVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILY
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	50 kDa
Gene Name	branched chain keto acid dehydrogenase E1, alpha polypeptide
Database Link	NP_000700 Entrez Gene 593 Human UniProt ID P12694
Background	The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]
Synonyms	BCKDE1A; MSU; MSUD1; OVD1A
Note	Immunogen Sequence Homology: Dog: 100%; Pig: 100%; Rat: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Sheep: 100%; Bovine: 100%; Rabbit: 100%; Guinea pig: 100%; Zebrafish: 93%
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Metabolic pathways, Valine, leucine and isoleucine degradation

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