|1.||Sánchez-Alcudia, R: 1 article (08/2014)|
|2.||Pérez-Cerdá, C: 1 article (08/2014)|
|3.||Desviat, L R: 1 article (08/2014)|
|4.||Navarrete, R: 1 article (08/2014)|
|5.||Ugarte, M: 1 article (08/2014)|
|6.||Pérez, B: 1 article (08/2014)|
|7.||Pérez-Carro, R: 1 article (08/2014)|
|8.||Pan, Guoyu: 1 article (03/2014)|
|9.||Hu, Zhenlei: 1 article (03/2014)|
|10.||Cheng, Xin: 1 article (03/2014)|
09/01/2013 - "Fumarylacetoacetate inhibits the initial step of the base excision repair pathway: implication for the pathogenesis of tyrosinemia type I."
09/01/1981 - "Fumarylacetoacetate fumarylhydorlase activity in liver tissue from a patient with hereditary tyrosinemia was found to be less 2% of the controls. "
08/15/2001 - "Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability."
09/01/2013 - "Hereditary tyrosinemia type I (HT1) is an autosomal recessive disease caused by a deficiency in human fumarylacetoacetate (FAA) hydrolase (FAH), which is the last enzyme in the catabolic pathway of tyrosine. "
02/15/2011 - "(6) Fumarylacetoacetate hydrolase (FAH) deficiency (tyrosinemia type I) may lead to hypermethioninemia secondary either to liver damage and/or to accumulation of fumarylacetoacetate, an inhibitor of the high K(m) MAT. "
|2.||Hepatocellular Carcinoma (Hepatoma)
01/01/1996 - "The reasons for the high incidence of hepatocellular carcinoma are unknown but it has been suggested that it may be caused by accumulated metabolites such as fumarylacetoacetate (FAA) and maleylacetoacetate (MAA). "
01/17/2012 - "The enzymatic defect impairs the conversion of fumarylacetoacetate to fumarate, causing accumulation of succinylacetone which induces severe liver and kidney dysfunction along with mutagenic changes and hepatocellular carcinoma. "
01/01/1990 - "This suggests that the development of hepatoma, which is frequent in this syndrome, and the presence of dysplastic changes of hepatocytes in nontumorous liver are related to genetic instability caused by accumulation of intermediates of tyrosine catabolism, which are natural alkylating agents (e.g., maleylacetoacetate and fumarylacetoacetate). "
|4.||Rare Diseases (Rare Disease)
08/01/2014 - "Hereditary tyrosinemia type I (HT1) is a rare disease caused by a deficiency of fumarylacetoacetate hydrolase (FAH) in the tyrosine catabolic pathway, resulting mainly in hepatic alterations due to accumulation of the toxic metabolites fumarylacetoacetate, maleylacetoacetate and succinylacetone. "
|5.||Acute Liver Failure (Fulminant Hepatic Failure)
|1.||fumarylacetoacetase (fumarylacetoacetate hydrolase)
|4.||Cytochromes c (Cytochrome c)
|5.||Tyrosine Transaminase (Tyrosine Aminotransferase)
|1.||Transplantation (Transplant Recipients)