Analytical Data
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基因名
ACADVL
- Application
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别名
ACADVL；VLCAD；Very long-chain specific acyl-CoA dehydrogenase. mitochondrial
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种属
Human
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表达系统
E. coli
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标签
His tag N-Terminus
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纯度
Greater than 90% as determined by SDS-PAGE.
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蛋白编号
P49748
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表达区间
41-655aa
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氨基酸序列
MGSSHHHHHHSSGLVPRGSHMAGGAAQLALDKSDSHPSDALTRKKPAKAE SKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDP AKNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGM HDLGVGITLGAHQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEP SSGSDAASIRTSAVPSPCGKYYTLNGSKLWISNGGLADIFTVFAKTPVTD PATGAVKEKITAFVVERGFGGITHGPPEKKMGIKASNTAEVFFDGVRVPS ENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQF GEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISK IFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILR LFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGL SLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLA DGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMA ALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
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分子量
69 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
ACADVL, or Acyl-CoA Dehydrogenase, Very Long Chain, is a critical enzyme involved in the mitochondrial fatty acid oxidation pathway, particularly responsible for the breakdown of very long-chain fatty acids (VLCFAs). Deficiency in ACADVL leads to the accumulation of VLCFAs, resulting in a metabolic disorder known as very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), which can cause severe clinical symptoms, including hypoketotic hypoglycemia, muscle weakness, and cardiomyopathy. The study of recombinant ACADVL protein has gained significant attention in the field of metabolic disorders, as it provides insights into the enzyme's structure and function, enabling researchers to explore its catalytic mechanism and regulation. Advances in molecular biology techniques, including cloning and expression systems, have facilitated the production of recombinant ACADVL, allowing scientists to investigate its biochemical properties and interactions with substrates and inhibitors. Understanding the folding and stability of the recombinant protein can also lead to identifying potential therapeutic strategies for VLCADD, including enzyme replacement therapies. Furthermore, investigating the implications of ACADVL functionality at the molecular level may elucidate broader metabolic pathways affected by VLCFA metabolism, highlighting its significance not only in clinical contexts but also in understanding lipid metabolism regulation in health and disease. Therefore, research on ACADVL recombinant proteins contributes vital information to both basic science and clinical applications, aiming to improve diagnostics and treatment for patients with VLCADD and related metabolic disorders.
