产品类别

联系我们

电话:025-68037686

地址:江苏生命科技创新园F6幢1层

订购:nanjing03@biogot.com

服务:biorase01@biogot.com

合作:lvyun@biogot.com

支持:may@biogot.com

FAH polyclonal antibody

货号: BS8270

价格：￥ 1140

货期：现货（1-2个工作日）

25ul 50ul 100ul 200ul

抗体定制服务咨询

说明书 MSDS SHIP

详细信息检测数据实验指南文献

No. :BS8270

Product Name :FAH polyclonal antibody

Swiss-Prot :P16930

Host :Rabbit

Reactivity :Human,Mouse,Rat

Applications :WB IHC

Application_all :WB: 1:500~1:2000 IHC: 1:50~1:200

Background :Fumarylacetoacetase is a 419 amino acid protein encoded by the human gene FAH. Fumarylacetoacetase catalyzes the hydrolysis of 4-fumarylacetoacetate, an intermediate in the metabolism of tyrosine, into acetoacetate and fumarate. Defects in FAH are the cause of tyrosinemia type I. It 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. Tyrosinemia type I is a rare condition, except in the Saguenay-lac-St-Jean region (province of Quebec, Canada) where the frequency is 1/1,846 newborns as the result of a founder effect.

Product :Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Purification&Purity :The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity :FAH polyclonal antibody detects endogenous levels of FAH protein.

BiowMW :~ 46 kDa

Note :For research use only, not for use in diagnostic procedure.

Alternative Name :Fumarylacetoacetase; FAA; Beta-diketonase; Fumarylacetoacetate hydrolase; FAH

Immunogen :Recombinant full length Human FAH.

Conjugate :Unconjugated

Modification :Unmodification

WesternBlot (WB) analysis of FAH polyclonal antibody

免疫沉淀（IP）

免疫组织化学（IHC）

免疫荧光（IF）