延胡索酰乙酰乙酸水解酶抗体
规格：1mg/1ml
英文名： Fumarylacetoacetate hydrolase
别名： Beta diketonase; FAA; FAAA_HUMAN; FAH; Fumarylacetoacetase; Fumarylacetoacetate; Fumarylacetoacetate hydrolase.
分子量： 47kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Fumaryla
交叉反应：Human, Mouse, Rat, Pig,
细胞定位：
延胡索酰乙酰乙酸水解酶抗体产品介绍：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延胡索酰乙酰乙酸水解酶抗体 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. Gene ID:延胡索酰乙酰乙酸水解酶抗体 2184 Database links: Entrez Gene: 2184 Human Entrez Gene: 14085 Mouse Entrez Gene: 29383 Rat Omim: 613871 Human SwissProt: P16930 Human SwissProt: P35505 Mouse SwissProt: P25093 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
延胡索酰乙酰乙酸水解酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 细胞骨架 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid