产品名称
ACADL Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/33
Human Swissprot No.
P28330
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P28330/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/11363
Mouse Swissprot No.
P51174
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/P51174
Rat Gene Link
https://www.uniprot.org/uniprot/25287
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/P15650
免疫原
Synthesized peptide derived from human ACADL AA range: 258-308
特异性
This antibody detects endogenous levels of ACADL at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
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, Jul 2008],
细胞定位
Mitochondrion matrix .
功能
catalytic activity:Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.,cofactor:FAD.,disease:Impaired ACADL activity results in non-ketotic hypoglycemia, hypotonia, muscle weakness and episodes of cardiorespiratory arrest associated with fasting [MIM:201460].,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homotetramer.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
