U-Blot® ACADV Rabbit mAb
Ucallm_WR6042-50
Price range: $268.00 through $328.00
Product details
Background：
acyl-CoA dehydrogenase , very long chain (ACADVL) Homo sapiens The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq , Jul 2008] ,
Specifications
| Size | 50μL/100μL |
| Target | ACADV |
| Reactivity | Human |
| Applications | WB, IHC, IF, IP, ELISA |
| MW(Calculated) | 70kD |
| MW(Observed) | 67kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant 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 | |
| Clonality | Monoclonal |
| Clone Number | RM2014R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | ACADVL VLCAD |
| Protein Name | Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9) |
| Other Name | |
| Species | Human |
| Gene ID | 37 |
| Uniprot | P49748, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | P50544, |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | P45953 |
| Cellular Localization | Mitochondrion inner membrane; Peripheral membrane protein .; [Isoform 2]: Mitochondrion inner membrane; Peripheral membrane protein . |
| Tissue Expression | Adipose tissue,Liver,Lung,Pancreas,Peripheral blood,Placenta, |
| Signaling_pathway | |
| Research Areas | >>Fatty acid degradation; / >>Metabolic pathways; / >>Fatty acid metabolism; / >>Alcoholic liver disease |
| Function | Catalytic activity:Acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.,cofactor:FAD.,Disease:Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.,Function:Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.,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:Homodimer., |
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