Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human heart muscle tissue showing cytoplasmic staining of myocytes.Application
Western blot analysis of ACADVL expression in non-transfected: (A) and human ACADVL transfected: (B) 293T whole cell lysates.Application
Western blot analysis of ACADVL expression in 293T whole cell lysate.| Product Name | ACADVL Antibody (H-110) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 472-580 mapping near the C-terminus of ACADVL of human origin |
| Modification Notes | ACADVL (acyl-Coenzyme A dehydrogenase, very long chain), also known as VLCAD, LCACD or ACAD6, is an inner mitochondrial membrane protein that belongs to the family of acyl-CoA dehydrogenases. Involved in lipid metabolism, ACADVL has catalytic activity toward esters of long chain and very long chain fatty acids and functions in the first step of the fatty acid beta-oxidation pathway. Defects in the gene encoding ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency), an autosomal recessive disease that leads to impaired long-chain fatty acid oxidation. VLCAD deficiency can give rise to three different phenotypes: a severe childhood form with a high incidence of mortality and cardiomyopathy; a mild childhood form with hypoketotic hypoglycemia and low mortality rates; and an adult form characterized by myoglobinuria and rhabdomyolysis. Two isoforms of ACADVL exist due to alternative splicing events. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of ACADVL of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 472-580 mapping near the C-terminus of ACADVL of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 472-580 mapping near the C-terminus of ACADVL of human origin |
| Entrez Gene | 37 |
|---|
Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human heart muscle tissue showing cytoplasmic staining of myocytes.
Application
Western blot analysis of ACADVL expression in non-transfected: (A) and human ACADVL transfected: (B) 293T whole cell lysates.
Application
Western blot analysis of ACADVL expression in 293T whole cell lysate.| Application Notes | recommended for detection of ACADVL of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
