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ACADV Monoclonal Antibody

BYmab-07315

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3个工作日

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产品简介>

宿主
反应性
应用
WB
分子量(DA)
72kD
免疫原
Synthesized peptide derived from human protein . at AA range: 471-520
特异性
ACADV Monoclonal Antibody detects endogenous levels of protein.
来源
Monoclonal, Mouse,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
稀释比例
WB 1:500-2000
纯化工艺(Immunogen)
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
背景(Background)
acyl-CoA dehydrogenase, very long chain(ACADVL) Homo sapiens The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008],
功能
catalytic activity:Acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.,cofactor:FAD.,disease:Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.,function:Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-
基因名称(Gene Name)
ACADVL VLCAD
蛋白名称
Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9)
简称
ACADV
其他名称
Fields
>>Fatty acid degradation;>>Metabolic pathways;>>Fatty acid metabolism;>>Alcoholic liver disease
人基因ID
人蛋白质序列数据库
小鼠基因ID
小鼠蛋白质序列数据库
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein .; [Isoform 2]: Mitochondrion inner membrane ; Peripheral membrane protein .
组织表达
Adipose tissue,Liver,Lung,Pancreas,Peripheral blood,Placenta,
储存(Storage)
-20°C/1 year

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ACADV Monoclonal Antibody

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