|Immunohistochemistry (Paraffin) (IHC (P))||1:100-1:1000|
|Western Blot (WB)||1:500-1:3000|
|Tested Species reactivity||Human|
|Host / Isotype||Rabbit / IgG|
|Immunogen||Recombinant fragment corresponding to a region within amino acids 252 and 445 of Human BCKDHA|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS, pH 7, with 1% BSA, 20% glycerol|
|Storage conditions||-20° C, Avoid Freeze/Thaw Cycles|
PA5-29300 targets BCKDHA in IF, IHC (P), and WB applications and shows reactivity with Human samples.
The PA5-29300 immunogen is recombinant fragment corresponding to a region within amino acids 252 and 445 of Human BCKDHA.
The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 126.96.36.199), 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 [PubMed 9582350]). 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; MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3; MIM 238331). E1 is a thiamine pyrophosphate (TPP)-dependent enzyme. The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The BCKDHB gene (MIM 248611) encodes the beta subunit of E1. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; branched chain keto acid dehydrogenase E1 alpha protein; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; mitochondrial; MSU; MSUD1; OVD1A
Gene Aliases: BCKDE1A; BCKDHA; MSU; MSUD1; OVD1A
UniProt ID: (Human) P12694
Entrez Gene ID: (Human) 593
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