BCKDHA Polyclonal Antibody | Invitrogen PA5-29300 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

Invitrogen

other brands :

NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience

product type :

antibody

product name :

BCKDHA Polyclonal Antibody

catalog :

PA5-29300

quantity :

100 uL

price :

US 514.00

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunohistochemistry, immunocytochemistry, immunohistochemistry - paraffin section

citations: 1

Reference
Billington C, Chapman K, Leon E, Meltzer B, Berger S, Olson M, et al. Genomic and biochemical analysis of repeatedly observed variants in DBT in individuals with maple syrup urine disease of Central American ancestry. Am J Med Genet A. 2022;188:2738-2749 pubmed publisher

product information

Product Type :

Antibody

Product Name :

BCKDHA Polyclonal Antibody

Catalog # :

PA5-29300

Quantity :

100 uL

Price :

US 514.00

Clonality :

Polyclonal

Purity :

Antigen affinity chromatography

Host :

Rabbit

Reactivity :

Human

Applications :

Immunocytochemistry: 1:100-1:1,000, Immunohistochemistry (Paraffin): 1:100-1:1,000, Western Blot: 1:500-1:3,000

Species :

Human

Isotype :

IgG

Storage :

Store at 4 C short term. For long term storage, store at -20 C, avoiding freeze/thaw cycles.

Description :

The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of the branched-chain alpha-ketoacids derived from isoleucine, leucine, and valine. This reaction is the second major step in the catabolism of the branched-chain amino acids (Wynn et al., 1998). The BCKD complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2), and a homodimeric dihydrolipoamide dehydrogenase (E3). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.

Immunogen :

Recombinant fragment corresponding to a region within amino acids 252 and 445 of Human BCKDHA

Format :

Liquid

Applications w/Dilutions :

Immunocytochemistry: 1:100-1:1,000, Immunohistochemistry (Paraffin): 1:100-1:1,000, Western Blot: 1:500-1:3,000

Aliases :

2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKAD E1[a]; BCKDA; BCKDE1A; BCKDH E1-alpha; BCKDHA; branched chain alpha-ketoacid dehydrogenase subunit E1 alpha; branched chain keto acid dehydrogenase E1 alpha protein; branched chain keto acid dehydrogenase E1, alpha polypeptide; branched chain keto acid dehydrogenase subunit E1, alpha polypeptide; branched chain ketoacid dehydrogenase E1, alpha polypeptide; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; E1a; MSU; MSUD1; OVD1A