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fumarylacetoacetate

National Institutes of Health

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2 relations

Broader (1)

Acetoacetates
Fumarylacetoacetate:MCnc:Pt:Ser:Qn

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2016
2016
Novel role of a triglyceride-synthesizing enzyme: DGAT1 at the crossroad between triglyceride and cholesterol metabolism
2010
2010
Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways.
2007
2007
Slow-onset inhibition of fumarylacetoacetate hydrolase by phosphinate mimics of the tetrahedral intermediate: kinetics, crystal structure and pharmacokinetics.
FAH (fumarylacetoacetate hydrolase) catalyses the final step of tyrosine catabolism to produce fumarate and acetoacetate. HT1… 
2004
2004
X-ray structure of fumarylacetoacetate hydrolase family member Homo sapiens FLJ36880
Abstract The human protein FLJ36880 belongs to the fumarylacetoacetate hydrolase family. The X-ray structure of FLJ36880 has been… 
2003
2003
Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC).
2001
2001
Structural and Functional Analysis of Missense Mutations in Fumarylacetoacetate Hydrolase, the Gene Deficient in Hereditary Tyrosinemia Type 1*
Hereditary tyrosinemia type 1 (HT1) is an autosomal recessive disease caused by a deficiency of the enzyme involved in the last… 
Highly Cited
1993
Highly Cited
1993
Rescue of mice homozygous for lethal albino deletions: implications for an animal model for the human liver disease tyrosinemia type 1.
Mice homozygous for specific deletions around the albino locus on chromosome 7 die within the first few hours of birth. They have… 
Highly Cited
1981
Highly Cited
1981
Deficiency of fumarylacetoacetase in a patient with hereditary tyrosinemia.
1977
1977
Characterization and substrate specificity of fumarylacetoacetate fumarylhydrolase.
The molecular weight of fumarylacetoacetate fumarylhydrolase (EC 3.7.1.2) is 86 000 +/- 10 000, as determined by gel filtration… 
1951
1951
Transamination as a step in tyrosine metabolism.
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