Analytical Data
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Gene name
BCKDHA
- Application
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Alternative Names
BCKDHA；2-oxoisovalerate dehydrogenase subunit alpha. mitochondrial
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Species
Human
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Source
E. coli
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Tag
His tag N-Terminus
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
P12694
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Expression Region
46-445aa
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AA 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
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Molecular Weight
48 kDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
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Protein Description
BCKDHA, or Branched-Chain α-Keto Acid Dehydrogenase E1 Alpha Subunit, is a critical enzyme in the catabolism of branched-chain amino acids (BCAAs) like leucine, isoleucine, and valine. Mutations in the BCKDHA gene can lead to Maple Syrup Urine Disease (MSUD), a rare but severe metabolic disorder that results in the accumulation of toxic metabolites in the body, causing neurological damage and developmental issues. Given the importance of BCAAs in protein synthesis and energy metabolism, understanding the structure and function of BCKDHA is essential for developing potential therapeutic strategies. Recombination techniques allow for the production of a functional BCKDHA protein, facilitating in-depth biochemical studies, structural characterization, and the investigation of its enzymatic activity. Research on the recombinant BCKDHA protein can provide insights into its mechanism of action, interactions with other metabolic pathways, and the effects of specific mutations, ultimately aiding in the design of targeted therapies for MSUD and other metabolic disorders. Moreover, this research could lead to the identification of biomarkers for early diagnosis and improved understanding of BCAA metabolism's role in various physiological processes and diseases.
