SKU: WR4421 Categories: ,

U-Blot® CPT1A Rabbit mAb

Price range: $268.00 through $328.00

SKU: WR4421-50
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Product details

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],

Specifications

Size50μL/100μL
TargetCPT1A
ReactivityHuman, Mouse, Rat, Duck
ApplicationsWB, IHC, IF, ELISA
MW(Calculated)88kD
MW(Observed)88kD
Host SpeciesRabbit
IsotypeIgG,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioIHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRM0640R
ImmunogenThe specific immunogen used to produce this antibody is proprietary information.
SpecificityEndogenous
Gene NameCPT1A CPT1
Protein NameCPT1A
Other NameCarnitine O-palmitoyltransferase 1, liver isoform; / CPT1-L; / Carnitine O-palmitoyltransferase I, liver isoform; / CPT I; / CPTI-L; / Carnitine palmitoyltransferase 1A
SpeciesHuman
Gene ID1374
UniprotP50416,
Species.1Mouse
Gene ID.112894
Uniprot.1P97742,
Species.2Rat
Gene ID.225757
Uniprot.2P32198
Cellular LocalizationMitochondrion outer membrane
Tissue ExpressionStrong expression in kidney and heart, and lower in liver and skeletal muscle.
Signaling_pathwayOrganismal Systems >> Endocrine system >> Glucagon signaling pathway;Organismal Systems >> Endocrine system >> Adipocytokine signaling pathway;Organismal Systems >> Endocrine system >> PPAR signaling pathway;Environmental Information Processing >> Signal transduction >> AMPK signaling pathway
Research Areas>>Fatty acid degradation; / >>Fatty acid metabolism; / >>PPAR signaling pathway; / >>AMPK signaling pathway; / >>Thermogenesis; / >>Adipocytokine signaling pathway; / >>Glucagon signaling pathway; / >>Insulin resistance; / >>Alcoholic liver disease
FunctionCatalytic 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.,
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