产品名称
CPT1A Rabbit Polyclonal Antibody
别名
Carnitine O-palmitoyltransferase 1, liver isoform (CPT1-L) (EC 2.3.1.21) (Carnitine O-palmitoyltransferase I, liver isoform) (CPT I) (CPTI-L) (Carnitine palmitoyltransferase 1A)
反应种属
Human,Mouse,Rat,Canine
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.346% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/1374
Human Swissprot No.
P50416
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P50416/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/12894
Mouse Swissprot No.
P97742
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/P97742
Rat Gene Link
https://www.uniprot.org/uniprot/25757
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/P32198
免疫原
Synthesized peptide derived from human CPT1A. AA range 40-80
特异性
This antibody detects endogenous levels of CPT1A at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008],
组织表达
Strong expression in kidney and heart, and lower in liver and skeletal muscle.
细胞定位
Mitochondrion outer membrane ; Multi-pass membrane protein .
功能
catalytic activity:Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.,disease:Defects in CPT1A are the cause of carnitine palmitoyltransferase I deficiency (CPT-I deficiency) [MIM:255120]; also known as CPT1A deficiency. CPT I deficiency is a rare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood.,enzyme regulation:Inhibitors such as malonyl-CoA interact with its catalytic domain and not with an associated regulatory component.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:Belongs to the carnitine/choline acetyltransferase family.,tissue specificity:Strong expression in kidney and heart, and lower in liver and skeletal muscle.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
