產品編號 | bs-17189R |
英文名稱 | Rabbit Anti-IVD antibody |
中文名稱 | 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. |
研究領域 | 腫瘤 細胞生物 神經生物學 信號轉導 新陳代謝 干擾素 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應 | Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse) |
產品應用 | 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 |
產品圖片 | |
1、抗體溶解方法 | |
2、抗體修復方式 | |
3、常用試劑的配制 | |
4、免疫組化操作步驟 | |
5、免疫組化問題解答 | |
6、Western Blotting 操作步驟 | |
7、Western Blotting 問題解答 | |
8、關于肽鏈的設計 | |
9、多肽的溶解與保存 | |
10、酶標抗體效價測定程序 | |