BCKDHA Antibody
Product Sizes
100ul
£630.00
OAGA03790-100UL
About this Product
- SKU:
- OAGA03790
- Additional Names:
- MSU; MSUD1; OVD1A; BCKDE1A
- Clonality:
- Polyclonal
- Concentration:
- 1 mg/ml
- Extra Details:
- 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.
- Gene Details:
- branched chain keto acid dehydrogenase E1; alpha polypeptide
- Host:
- Rabbit
- Immunogen:
- Recombinant protein encompassing a sequence within the C-terminus region of human BCKDHA. The exact sequence is proprietary.
- Isotype:
- IgG
- Protein Details:
- 2-oxoisovalerate dehydrogenase subunit alpha; mitochondrial
- Purification:
- Purified by antigen-affinity chromatography.
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- Keep as concentrated solution. Aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.
- Supplier:
- Aviva Systems Biology
- Type:
- Antibodies: Polyclonal Antibody
- Manufacturer's Data Sheet:html_datasheet.php
