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上海岚派生物科技有限公司 > 产品类别 > 抗体 > 标记一抗 >
FITC标记的酰基辅酶A脱氢酶长链抗体
- 品名：
- FITC标记的酰基辅酶A脱氢酶长链抗体
- 货号：
- DL-1187
- 英文：
- Anti-ACADL/FITC
- 英文缩写：
background:
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq].
Subunit:
Homotetramer.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.
Similarity:
Belongs to the acyl-CoA dehydrogenase family.
