| 產(chǎn)品編號 | bs-16194R |
| 英文名稱 | Rabbit Anti-Fumarylacetoacetate hydrolase antibody |
| 中文名稱 | 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. |
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Specific References (1) | bs-16194R has been referenced in 1 publications.
[IF=2.634] Gao M et al. Efficient?Generation?of an?Fah/Rag2?Dual-Gene?Knockout?Porcine?Cell?Line?Using?CRISPR/Cas9and?Adenovirus. DNA Cell Biol. 2019 Feb 14. ICC&IF ; Porcine.
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| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 細(xì)胞骨架 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Pig) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 47 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞外基質(zhì) 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Fumarylacetoacetate hydrolase: 21-120/419 |
| 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 |
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. SWISS: P16930 Gene ID: 2184 Database links: Entrez Gene: 2184 Human Entrez Gene: 14085 Mouse Omim: 613871 Human SwissProt: P16930 Human SwissProt: P35505 Mouse |
