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P28330?
Protein names
?Long-chain specific acyl-CoA dehydrogenase, mitochondrial
LCAD
蛋白中文名
?长链特异酰基辅酶A脱氢酶, 线粒体
?隐藏详细信息
Organism
?Homo sapiens
Gene ID
?33
Taxonomic identifier
?9606
Entry name
?ACADL_HUMAN
Gene names
?ACADL
Taxonomic lineage
?Eukaryota -> Metazoa -> Chordata -> Craniata -> Vertebrata -> Euteleostomi -> Mammalia -> Eutheria -> Euarchontoglires -> Primates -> Haplorrhini -> Catarrhini -> Hominidae -> Homo
Sequence length
?430
Sequence status
?Complete.
Protein existence
?Evidence at transcript level
Subunit structure
?Homotetramer.
Subcellular location
?Mitochondrion matrix.
Miscellaneous
?A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
Sequence similarities
?Belongs to the acyl-CoA dehydrogenase family.
Involvement in 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.
Pathway
?Lipid metabolism; mitochondrial fatty acid beta- oxidation.
Catalytic activity
?Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.
Cofactor
?FAD.
Biological process
?Fatty acid metabolism; Lipid metabolism
Cellular component
?Mitochondrion
Coding sequence diversity
?Polymorphism
[KW] Domain
?Transit peptide
Ligand
?FAD; Flavoprotein
Molecular function
?Oxidoreductase
Technical term
?Complete proteome
Gene Ontology Biological process
?temperature homeostasis
regulation of cholesterol metabolic process
Gene Ontology Cellular component
?mitochondrial matrix
Gene Ontology Molecular function
?

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公司名称产品编号产品名称包装价格详细信息
Abnova? 查看代理商?H00000033-R01?ACADL Pre-design Chimera RNAi?10 nmol?$220.00
Abnova? 查看代理商?H00000033-R01?ACADL Pre-design Chimera RNAi?20 nmol?$264.00
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Abnova? 查看代理商?H00000033-A01?ACADL polyclonal antibody (A01)
?ACADL polyclonalyabo狗亚体育 (A01)
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Abgent? 查看代理商?AP8536c?ACADL Antibody (Center)
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Abgent? 查看代理商?RI10040?ACADL predesign siRNA??
Abcam? 查看代理商?ab82853?ACADL antibody
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Novus? 查看代理商?H00000033-A01?ACADL Antibody
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Novus? 查看代理商?H00000033-P01?ACADL Recombinant Protein
?ACADL重组蛋白
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Novus? 查看代理商?H00000033-R01?ACADL RNAi??
Avivasysbio? 查看代理商?ARP33856_P050?ACADL (acyl-Coenzyme A dehydrogenase, long chain) Antibody (against the middle region of ACADL)
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Avivasysbio? 查看代理商?ARP33855_P050?ACADL (acyl-Coenzyme A dehydrogenase, long chain) Antibody (against the N terminal of ACADL)
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Avivasysbio? 查看代理商?AAPP04926?ACADL (acyl-Coenzyme A dehydrogenase, long chain) Blocking Peptide
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Avivasysbio? 查看代理商?AAPP04927?ACADL (acyl-Coenzyme A dehydrogenase, long chain) Blocking Peptide (the middle region of protein)
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