The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Detects a band of approximately 64 kDa (predicted molecular weight: 64 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
功能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.
疾病相关Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:201475]. ACADVLD 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.
序列相似性Belongs to the acyl-CoA dehydrogenase family.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human pineal tissue labelling ACADVL with ab81497 at 1/100. A Cy3-conjugated donkey anti-rabbit IgG (1/200) was used as the secondary antibody. Positive staining shown in the cytoplasm of cell bodies of pinealocytes. Magnification: 20X. Exposure time: 0.5 - 2.0 seconds. Left - DAPI. Middle - ACADVL. Right - Merge.
Western blot - ACADVL antibody (ab81497)
Anti-ACADVL antibody (ab81497) at 1 µg/ml (in 5% skim milk / PBS buffer) + HepG2 cell lysate at 10 µg
Secondary HRP conjugated anti-Rabbit IgG at 1/50000 dilution
Predicted band size : 64 kDa Observed band size : 64 kDa Gel concentration 12%
Anti-ACADVL antibody (ab81497)参考文献
has not yet been referenced specifically in any publications.