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
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.346% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（Background）
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],
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.,
基因名称（Gene Name）
CPT1A CPT1
其他名称
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)
Fields
>>Fatty acid degradation;>>Fatty acid metabolism;>>PPAR signaling pathway;>>AMPK signaling pathway;>>Thermogenesis;>>Adipocytokine signaling pathway;>>Glucagon signaling pathway;>>Insulin resistance;>>Alcoholic liver disease
细胞定位
Mitochondrion outer membrane ; Multi-pass membrane protein .
组织表达
Strong expression in kidney and heart, and lower in liver and skeletal muscle.
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