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.
Locations
Orders Processing,
Shipping & Receiving,
Warehouse
2 Shaker Rd Suites
B001/B101
Shirley, MA 01464
Production Lab
Floor 6, Suite 620
20700 44th Avenue W
Lynnwood, WA 98036
Telephone Numbers
Tel: +1 (206) 374-1102
Fax: +1 (206) 577-4565
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)
Your search did not match any products.
If you do not find the reagent or information you require, please contact Customer.Support@LSBio.com to inquire about additional products in development.
