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human BCKDHA Adenovirus

Name 
Ad-h-BCKDHA
Cat No 
ADV-202026
Availability
4-5 weeks
Categories
Adenovirus, Over-Expression

This is an Adenovirus expressing Human BCKDHA.

ADV-202026
Ad-h-BCKDHA

Ready-to-use Adenovirus expressing Human BCKDHA (branched chain keto acid dehydrogenase E1, alpha polypeptide). Available with optional GFP reporter or cell-specific promoter.

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Product Details

Promoter
CMV
Reporter
none, optional GFP, CFP, YFP, RFP or mCherry
Storage Buffer
DMEM, 2% BSA, 2.5% Glycerol
Viral Backbone
Human Adenovirus Type5 (dE1/E3)

Gene Reference Data

Alternate Names
2-oxoisovalerate dehydrogenase (lipoamide); 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; MSU; MSUD1; OVD1A
Description (eCommerce)
BCKDHA (branched chain keto acid dehydrogenase E1, alpha polypeptide) is a component of the branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex. The BCKD complex is an inner 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). Mutations in this gene result in maple syrup urine disease, type IA [taken from NCBI Entrez Gene (Gene ID: 593)].
Description (Vector)
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 ID
593
Gene Name (eCommerce)
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Name (Vector)
branched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Symbol
BCKDHA
HGNC ID
HGNC:986
NCBI Taxonomy ID (eCommerce)
9606.0
ORF Size (aa)
1338
ORF Size (bp)
1338 bp
Protein Name (eCommerce)
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
RefSeq ID
NM_000709
RefSeq Synonyms
NM_001164783, NM_000709, BC023983, BC008933, BC007878,
Research Areas
Metabolism/Metabolic Process,Mitochondrion
Research Areas (Faceted)
cell_biology,metabolism
Species
human
Target Sentence
BCKDHA (branched chain keto acid dehydrogenase E1, alpha polypeptide) is a component of the branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex. The BCKD complex is an inner 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). Mutations in this gene result in maple syrup urine disease, type IA [taken from NCBI Entrez Gene (Gene ID: 593)].
UniGene ID
Hs.433307
UniProt ID (eCommerce)
P12694

Related Citations

About Storage Conditions

All our viral products should be kept at -80°C. At this temperature, the virus will remain stable for 6-12 months (and in some cases, up to 2 years). Once thawed, the product can be stored at 4°C for 2-3 weeks without significant loss of biological activity. We recommend aliquoting your vectors into low protein binding tubes upon receipt. This helps avoid repeated freeze-thaw cycles, as well as prevent loss of virus. To maintain accurate titer, aliquot in at least 20ul per tube.