Hereditary fructose intolerance syndrome

Known as: Fructose Intolerances, Hereditary, Deficiency, Fructose-1,6-Biphosphate Aldolase, Fructose 1 Phosphate Aldolase Deficiency 
An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation… (More)
National Institutes of Health

Papers overview

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Highly Cited
2012
Highly Cited
2012
Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2… (More)
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Highly Cited
2007
Highly Cited
2007
BACKGROUND A high fructose intake can lead to postprandial hypertriacylglycerolemia. The underlying mechanism is unclear… (More)
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Review
2006
Review
2006
  • M. Buse
  • American journal of physiology. Endocrinology and…
  • 2006
The hexosamine biosynthesis pathway (HBP) is a relatively minor branch of glycolysis. Fructose 6-phosphate is converted to… (More)
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Highly Cited
2000
Highly Cited
2000
BACKGROUND Orthostatic intolerance is a syndrome characterized by lightheadedness, fatigue, altered mentation, and syncope and… (More)
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Highly Cited
2000
Highly Cited
2000
We believe that the pattern of blood pressure response to tilt during the time preceding the development of the vasovagal… (More)
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Highly Cited
1991
Highly Cited
1991
Based on our previous finding that desensitization of the insulin-responsive glucose transport system (GTS) requires three… (More)
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Highly Cited
1991
Highly Cited
1991
BACKGROUND Endurance athletes have a high incidence of orthostatic intolerance. We hypothesized that this is related to an… (More)
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Highly Cited
1982
Highly Cited
1982
Pyrophosphate : fructose-6-phosphate phosphotransferase (PPi-PFK) has been purified 150-fold from potato tubers and the kinetic… (More)
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Highly Cited
1974
Highly Cited
1974
Mutant strains of Pseudomonas putida (arvilla) mt-2 which have lost the ability to grow at the expense of m- or p-toluate… (More)
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Highly Cited
1964
Highly Cited
1964
This is a record of the concentrations of the nonenzyme components of the Embden-Meyerhof system in mouse brain measured at brief… (More)
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