| 產(chǎn)品編號 | bs-16194R-Gold |
| 英文名稱(chēng) | Rabbit Anti-Fumarylacetoacetate hydrolase/Gold Conjugated antibody |
| 中文名稱(chēng) | 膠體金標記的延胡索酰乙酰乙酸水解酶抗體 |
| 別 名 | Beta diketonase; FAA; FAAA_HUMAN; FAH; Fumarylacetoacetase; Fumarylacetoacetate; Fumarylacetoacetate hydrolase. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul（10nm 15nm 35nm） |
| 研究領(lǐng)域 | 腫瘤 細胞生物 免疫學(xué) 細胞骨架 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | (predicted: Human, Mouse, Rat, Pig, ) |
| 產(chǎn)品應用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 47kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 0.4mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Fumarylacetoacetate hydrolase |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
| 保存條件 | Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles. |
| 產(chǎn)品介紹 |
background: This gene encodes the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). [provided by RefSeq, Jul 2008] Function: Fumarylacetoacetate hydrolase is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). This is an autosomal recessive inborn error of metabolism that occurs in both an acute and a chronic form. Clinical characteristics of the acute form include hepatic failure and death in infancy, whereas children with the chronic form have renal tubular dysfunction and hypophosphatemic rickets, progressive liver disease with development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment. Subunit: Homodimer. Tissue Specificity: Mainly expressed in liver and kidney. Lower levels are also detected in many other tissues. Similarity: Belongs to the FAH family. Database links: Entrez Gene: 2184 Human Entrez Gene: 14085 Mouse Omim: 613871 Human SwissProt: P16930 Human SwissProt: P35505 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
