| 產(chǎn)品編號 | bs-5018R |
| 英文名稱 | Rabbit Anti-ACADVL antibody |
| 中文名稱 | 酰基輔酶A脫氫酶很長鏈抗體 |
| 別 名 | ACAD 6; ACAD6; ACADV_HUMAN; Acadvl; Acyl CoA dehydrogenase very long chain; Acyl Coenzyme A dehydrogenase very long chain; LCACD; mitochondrial; Very long chain specific acyl CoA dehydrogenase; Very long chain specific acyl CoA dehydrogenase mitochondrial; Very long-chain specific acyl-CoA dehydrogenase; VLCAD. |
| 研究領(lǐng)域 | 腫瘤 心血管 細(xì)胞生物 免疫學(xué) 信號轉(zhuǎn)導(dǎo) 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Rat (predicted: Human,Mouse,Rabbit,Pig,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 66kDa |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ACADVL: 251-350/655 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
ACADVL (acyl-Coenzyme A dehydrogenase, very long chain) catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. It is specific to esters of long-chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA. Deficiencies in ACADVL are associated with reduced myocardial fatty acid beta-oxidation and cardiomyopathy. Function: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Subunit: Homodimer. Subcellular Location: Mitochondrion inner membrane. DISEASE: 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. Similarity: Belongs to the acyl-CoA dehydrogenase family. SWISS: P49748 Gene ID: 37 Database links: Entrez Gene: 37 Human Entrez Gene: 11370 Mouse Omim: 609575 Human SwissProt: P49748 Human SwissProt: P50544 Mouse Unigene: 437178 Human Unigene: 18630 Mouse Unigene: 33319 Rat |
| 產(chǎn)品圖片 |
Paraformaldehyde-fixed, paraffin embedded (rat adrenal gland); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ACADVL) Polyclonal Antibody, Unconjugated (bs-5018R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ACADVL) Polyclonal Antibody, Unconjugated (bs-5018R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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