Validation Data Gallery
Tested Applications
Recommended dilution
| Application | Dilution |
|---|---|
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
Product Information
85889-1-PBS targets ACADVL in WB, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse samples.
| Tested Reactivity | human, mouse |
| Host / Isotype | Rabbit / IgG |
| Class | Recombinant |
| Type | Antibody |
| Immunogen |
CatNo: Ag6016 Product name: Recombinant human ACADVL protein Source: e coli.-derived, T-HIS Tag: 6*His Domain: 312-655 aa of BC012912 Sequence: FDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF 相同性解析による交差性が予測される生物種 |
| Full Name | acyl-Coenzyme A dehydrogenase, very long chain |
| Calculated molecular weight | 70 kDa |
| Observed molecular weight | 60 kDa |
| GenBank accession number | BC012912 |
| Gene Symbol | ACADVL |
| Gene ID (NCBI) | 37 |
| Conjugate | Unconjugated |
| Form | |
| Form | Liquid |
| Purification Method | Protein A purification |
| UNIPROT ID | P49748 |
| Storage Buffer | PBS only{{ptg:BufferTemp}}7.3 |
| Storage Conditions | Store at -80°C. |
Background Information
ACADVL (Acyl-CoA Dehydrogenase, Very Long Chain) is a mitochondrial enzyme that catalyzes the initial step of β-oxidation for very long-chain fatty acids (VLCFAs, C14-C20). Deficiency of ACADVL causes very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common inherited disorder of mitochondrial fatty acid oxidation, characterized by hypoketotic hypoglycemia, cardiomyopathy, hepatopathy, and exercise-induced rhabdomyolysis. ACADVL is highly expressed in metabolically active tissues including cardiac muscle, skeletal muscle, and liver, where it plays a critical role in energy production during fasting and high-demand states. Mutations in ACADVL represent a significant diagnostic target for newborn screening and genetic counseling in metabolic disorders.