order histories, retained contact details for faster checkout, review submissions, and special promotions.
Forgot password?
order histories, retained contact details for faster checkout, review submissions, and special promotions.
Location
Corporate Headquarters
Vector Laboratories, Inc.
6737 Mowry Ave
Newark, CA 94560
United States
Telephone Numbers
Customer Service: (800) 227-6666 / (650) 697-3600
Contact Us
Additional Contact Details
order histories, retained contact details for faster checkout, review submissions, and special promotions.
Forgot password?
order histories, retained contact details for faster checkout, review submissions, and special promotions.
BCKDHA / BCKDE1A
branched chain keto acid dehydrogenase E1, alpha polypeptide
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 Name: | branched chain keto acid dehydrogenase E1, alpha polypeptide |
| Synonyms: | BCKDHA, BCKDH E1-alpha, BCKDE1A, MSU, MSUD1, OVD1A |
| Target Sequences: | NM_000709 NP_000700.1 P12694 |
Publications (1)
If you do not find the reagent or information you require, please contact Customer.Support@LSBio.com to inquire about additional products in development.
