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fumarylacetoacetase activity

Known as: 4-fumarylacetoacetate fumarylhydrolase activity, beta-diketonase activity, fumarylacetoacetate hydrolase activity 
Catalysis of the reaction: 4-fumarylacetoacetate + H(2)O = acetoacetate + fumarate + H(+). [EC:3.7.1.2, RHEA:10247]
National Institutes of Health

Papers overview

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2004
2004
Tyrosinaemia type 1 is an autosomal recessive disorder characterized by reduced fumarylacetoacetate hydrolase activity (McKusick… Expand
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2003
2003
Hereditary tyrosinemia is an inborn error of tyrosine metabolism due to an inherited deficiency of fumarylacetoacetate hydrolase… Expand
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1997
1997
Hereditary tyrosinemia 1 (HT1) is characterized by progressive liver damage, from infancy, and by a high risk for hepatocellular… Expand
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1994
1994
In six unrelated patients with hereditary tyrosinemia type 1 (HT1), three different disease-causing mutations were found by DNA… Expand
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1986
1986
Fumarylacetoacetase has been determined in fibroblasts, lymphocytes and/or liver tissue from 46 patients affected or presumed to… Expand
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1985
1985
Two healthy adults with low fumarylacetoacetase activity in fibroblasts and lymphocytes, one a compound heterozygote for the… Expand
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1984
1984
ABSTRACT: Metabolic and enzymatic studies in a patient with hereditary tyrosinemia demonstrated for the first time a deficiency… Expand
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1983
1983
Summary: Fumarylacetoacetate fumarylhydrolase (E.C.3.7.1.2.), a liver enzyme involved in tyrosine degradation, is shown to be… Expand
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1981
1981
Extensive metabolic and enzymatic studies in a patient with hereditary hepatorenal tyrosinemia demonstrated for the first time a… Expand
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1968
1968
1. A diketo acid hydrolase was purified about 100-fold from rat liver and some of its properties were studied. 2. Comparison of… Expand
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