BCKDHA

Alternative Names: Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain, BCKDH E1-alpha, BCKDHA, BCKDE1A, 2-oxoisovalerate dehydrogenase subunit alpha mitochondrial, OVD1A, MSU, MSUD1, FLJ45695.

BCKDHA is a key enzyme in the BCKD complex, responsible for the catabolism of branched-chain amino acids in mitochondria. It consists of 3 catalytic components: E1, E2, and E3. Mutations in the BCKDHA gene cause maple syrup urine disease, type IA.

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  • Sku: PSB-enz-090-1µg
  • Vendor: ProSpec-Tany
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Title

Product Details

Product Description

Recombinant Human Branched Chain keto Acid Dehydrogenase E1, Alpha is a protein belonging to the family of keto acid dehydrogenases. With a molecular weight of approximately 45 kDa, this enzyme plays a key role in the catabolism of branched-chain amino acids. Its function involves catalyzing the first step in the degradation of these amino acids, ultimately leading to the production of acetyl-CoA and other metabolites. This protein is essential for maintaining proper metabolic processes within the body.

Current Lead Time

7-14 Days

Product Specifications

Species
Human
Expression System E. coli
Purity 80.0% by SDS-PAGE
Activity
Not Available
Activity Assay
Animal Component Free (ACF) Yes
Molecular Weight
47.8 kDa
Structure Homodimer
Endotoxin Concentration
Not Available
Purification Method Not Available
Form
Frozen
Formulation The BCKDHA solution (0.25 mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 5mM DTT, 30% glycerol and 0.2M NaCl.

*For Research Use Only

Product Guarantee

If your product is within its expiration period, and has not performed according to expectations, fill out the sample return form. Return the unused sample frozen, with the auto generated packing slip to the address on the instructions. You will receive a full refund along with a replacement of the unused portion (inclusive of the failed test). Damages do not cover any additional costs incurred for failed experimentation. 

Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MSSLDDKPQF PGASAEFIDK LEFIQPNVIS GIPIYRVMDR QGQIINPSED PHLPKEKVLK LYKSMTLLNT MDRILYESQR QGRISFYMTN YGEEGTHVGS AAALDNTDLV FGQYREAGVL MYRDYPLELF MAQCYGNISD LGKGRQMPVH YGCKERHFVT ISSPLATQIP QAVGAAYAAK RANANRVVIC YFGEGAASEG DAHAGFNFAA TLECPIIFFC RNNGYAISTP TSEQYRGDGI AARGPGYGIM SIRVDGNDVF AVYNATKEAR RRAVAENQPF LIEAMTYRIG HHSTSDDSSA YRSVDEVNYW DKQDHPISRL RHYLLSQGWW DEEQEKAWRK QSRRKVMEAF EQAERKPKPN PNLLFSDVYQ EMPAQLRKQQ ESLARHLQTY GEHYPLDHFD K.

Storage Instruction

Reconstituted store at -20°C or -80°C.We recommend that single use aliquots are prepared to avoid freeze-thaw cycles.

Sample Prep Instruction

1. Thaw the vial on ice, spin briefly
2. Prepare single-use or stock aliquots. This stage will depend on your final application so please adapt as appropriate. All our proteins are supplied carrier protein-free. If compatible with your work and you are storing at lower concentrations (<50 ug/ml) adding carrier protein is highly advised (usually 1% w/v high purity BSA or equivalent – ensure all buffers are sterile-filtered).
3. Prepare single-use aliquots whenever possible to avoid freeze-thaw cycles which can damage the proteins and reduce bioactivity. Store aliquots at -70 °C (or -20 °C)
4. We recommend sterile filtering after dilution in media or the final working solution

References

Not Available

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BCKDHA

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