Analytical Data
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基因名
BCKDHB
- Application
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别名
BCKDE1B; BCKDH E1-beta; BCKDHB; Branched chain alpha ketoacid dehydrogenase E1 beta subunit
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种属
Human
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表达系统
E. coli
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标签
GST-tag at N-terminal
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纯度
Greater than 90% as determined by SDS-PAGE.
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蛋白编号
P21953
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表达区间
1-392aa
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氨基酸序列
MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKRLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
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分子量
68.86 kDa
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内毒素
< 1.0 EU per μg protein as determined by the LAL method.
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性状
Freeze-dried powder
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缓冲液
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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复溶方法
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- 个性化定制
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稳定性测试
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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保存条件 & 期限
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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运输条件
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
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.
