human BCKDHA Over-expression AAV

Cat. No: AAV-202026

Availability: 3-5 weeks

Name: AAV-h-BCKDHA

This is an AAV expressing Human BCKDHA.

Request a Quote

Please enter your email address and we'll be in touch with more information:

Viral Backbone: Recombinant AAV
AAV-ITR: AAV2
AAV Serotype: Available in AAV1, AAV2, AAV3, AAV5, AAV6, AAV8, AAV9, AAV-DJ, AAV-DJ8, AAV-DJ9 and other wildtype/synthetic AAV capsids
Promoter: CMV (default), or choose from 30 different ubiquitous or cell specific promoter
Reporter: none, optional GFP, CFP, YFP, RFP or mCherry
Storage Buffer: PBS/5% Glycerol

Species: Human
Gene Symbol: BCKDHA
Gene Name: branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene ID: 593
Gene Synonyms: BCKDE1A; MSU; MSUD1; OVD1A
ORF Size: 1338 bp
RefSeq#: BC007878
Gene Description: 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.
UniGene ID: Hs.433307
HGNC ID: HGNC:986