| 產(chǎn)品編號 | bs-17189R |
| 英文名稱(chēng) | Rabbit Anti-IVD antibody |
| 中文名稱(chēng) | 異戊酰輔酶A脫氫酶抗體 |
| 別 名 | ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial. |
| 研究領(lǐng)域 | 腫瘤 細胞生物 神經(jīng)生物學(xué) 信號轉導 新陳代謝 干擾素 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
| 產(chǎn)品應用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 43kDa |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human IVD: 201-300/423 |
| 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 |
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009] Function: Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Similarity: Belongs to the acyl-CoA dehydrogenase family. SWISS: P26440 Gene ID: 3712 Database links: Entrez Gene: 3712 Human Entrez Gene: 56357 Mouse Omim: 607036 Human SwissProt: P26440 Human SwissProt: Q9JHI5 Mouse Unigene: 513646 Human Unigene: 6635 Mouse Unigene: 147 Rat |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with IVD polyclonal antibody, unconjugated (bs-17189R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
|
