Analytical Data
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Gene name
BCKDHB
- Application
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Alternative Names
BCKDE1B; BCKDH E1-beta; BCKDHB; Branched chain alpha ketoacid dehydrogenase E1 beta subunit
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Species
Human
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Source
E. coli
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Tag
GST-tag at N-terminal
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
P21953
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Expression Region
1-392aa
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AA Sequence
MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKRLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
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Molecular Weight
68.86 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
Related Products
Protein Description
BCKDHB, or Branched-Chain Ketoacid Dehydrogenase E2 Component, is a crucial enzyme involved in the metabolism of branched-chain amino acids (BCAAs), namely leucine, isoleucine, and valine. Mutations in the BCKDHB gene can lead to Maple Syrup Urine Disease (MSUD), a metabolic disorder characterized by an inability to properly degrade BCAAs, resulting in their toxic accumulation. Understanding the structure and function of BCKDHB is essential for devising therapeutic strategies for MSUD and other related metabolic disorders. Recent advancements in recombinant protein technology have enabled the production of BCKDHB in a laboratory setting, allowing for detailed studies of its enzymatic activity, kinetics, and interactions with other mitochondrial enzymes. This research not only contributes to a better understanding of the biochemical pathways involved in amino acid metabolism but also opens avenues for potential gene therapy approaches and enzyme replacement therapies. The recombinant BCKDHB protein can serve as a valuable tool for screening inhibitors or activators that may help in managing MSUD and related conditions, ultimately aiming to improve patient outcomes and pave the way for novel therapeutic interventions.
