验证数据展示
经过测试的应用
| Positive WB detected in | HeLa cells, HepG2 cells, HEK-293T cells, A431 cells, L02 cells, mouse liver tissue |
| Positive IF/ICC detected in | A549 cells |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Western Blot (WB) | WB : 1:5000-1:50000 |
| Immunofluorescence (IF)/ICC | IF/ICC : 1:250-1:1000 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
产品信息
85889-1-RR targets ACADVL in WB, IF/ICC, ELISA applications and shows reactivity with human, mouse samples.
| 经测试应用 | WB, IF/ICC, ELISA Application Description |
| 经测试反应性 | human, mouse |
| 免疫原 |
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 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Recombinant |
| 产品类型 | Antibody |
| 全称 | acyl-Coenzyme A dehydrogenase, very long chain |
| 别名 | VLCAD, EC:1.3.8.9, Very long-chain specific acyl-CoA dehydrogenase, mitochondrial |
| 计算分子量 | 70 kDa |
| 观测分子量 | 60 kDa |
| GenBank蛋白编号 | BC012912 |
| 基因名称 | ACADVL |
| Gene ID (NCBI) | 37 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | P49748 |
| 储存缓冲液 | PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. |
| 储存条件 | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
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.
实验方案
| Product Specific Protocols | |
|---|---|
| IF protocol for ACADVL antibody 85889-1-RR | Download protocol |
| WB protocol for ACADVL antibody 85889-1-RR | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
