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货号： bs-16194R-FITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-16194R-FITC
- 英文名称
- Anti-Fumarylacetoacetate hydrolase/FITC
- 中文名称
- FITC标记的延胡索酰乙酰乙酸水解酶抗体
- 别 名
- Beta diketonase; FAA; FAAA_HUMAN; FAH; Fumarylacetoacetase; Fumarylacetoacetate; Fumarylacetoacetate hydrolase.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 细胞骨架 新陈代谢
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 47kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Fumarylacetoacetate hydrolase
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- 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: 2184Human
Entrez Gene: 14085Mouse
Entrez Gene: 29383Rat
Omim: 613871Human
SwissProt: P16930Human
SwissProt: P35505Mouse
SwissProt: P25093Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.