Anti-ACADVL monoclonal antibody (DCABH-277)
Mouse anti-Human ACADVL monoclonal antibody for ICC, IP, IHC-P
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Mouse, Rat, Cow, Human
Human liver mitocondria
ICC: 5 μg/ml; IHC-P: 1/250.
*Suggested working dilutions are given as a guide only. It is recommended that the user titrates the product for use in their own experiment using appropriate negative and positive controls.
ACADVL; acyl-CoA dehydrogenase, very long chain; acyl Coenzyme A dehydrogenase, very long chain; very long-chain specific acyl-CoA dehydrogenase, mitochondrial; ACAD6; LCACD
ACADVL (Acyl-CoA Dehydrogenase, Very Long Chain) is a Protein Coding gene. Diseases associated with ACADVL include vlcad deficiency and very long-chain acyl-coenzyme a dehydrogenase deficiency. Among its related pathways are Metabolism and Transport to the Golgi and subsequent modification. GO annotations related to this gene include flavin adenine dinucleotide binding and acyl-CoA dehydrogenase activity. An important paralog of this gene is ACAD8. 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.
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. Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]: An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Note=The disease is caused by mutations affecting the gene represented in this entry. The function about ACADVL antigen include acyl-CoA dehydrogenase activity; fatty-acyl-CoA binding; flavin adenine dinucleotide binding; long-chain-acyl-CoA dehydrogenase activity; very-long-chain-acyl-CoA dehydrogenase activity.
Activation of Chaperone Genes by XBP1(S), organism-specific biosystem; Activation of Chaperones by IRE1alpha, organism-specific biosystem; Beta oxidation of palmitoyl-CoA to myristoyl-CoA, organism-specific biosystem; Diabetes pathways, organism-specific biosystem; Disease, organism-specific biosystem; FOXA2 and FOXA3 transcription factor networks, organism-specific biosystem; Fatty Acid Beta Oxidation, organism-specific biosystem.
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Tyni, T; Paetau, A; et al. Mitochondrial fatty acid beta-oxidation in the human eye and brain: Implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. PEDIATRIC RESEARCH 56:744-750(2004).
ANTOZZI, C; GARAVAGLIA, B; et al. LATE-ONSET RIBOFLAVIN-RESPONSIVE MYOPATHY WITH COMBINED MULTIPLE ACYL-COENZYME-A DEHYDROGENASE AND RESPIRATORY-CHAIN DEFICIENCY. NEUROLOGY 44:2153-2158(1994).
Custom Antibody Labeling
We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody.