Fructose-1,6-Diphosphatase Deficiency
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   Fructose-1,6-Diphosphatase Deficiency


Fructose-1,6-Diphosphatase Deficiency

   An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

RELATED TERMS
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Autosomal
"Pertaining to a chromosome that is not a sex chromosome; relating to any one of the chromosomes save the sex chromosomes. People normally have 22 pairs of autosomes (44 autosomes) in each cell together with two sex chromosomes (X and Y in the male and XX in the female). "

Fructose
Fruit sugar.

Metabolism
The chemical activity that occurs in cells, releasing energy from nutrients, or using energy to create other substances, such as proteins.

Fructose-1,6-diphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Gluconeogenesis
The process by which GLUCOSE is formed from a non-carbohydrate source.

Hypoglycemia
Low levels of blood sugar.

Ketosis
A condition of having ketone bodies build up in body tissues and fluids. The signs of ketosis are nausea, vomiting, and stomach pain. Ketosis can lead to ketoacidosis.

Acidosis
An abnormal condition in the body in which excessive acid lowers the pH of the blood and body tissues.

Infant
A child under a year of age.

Fasting
Abstaining from all food.

Febrile
Caused by fever. Feverish.

Childhood
1. The time for a boy or girl from birth until he or she is an adult. 2. The more circumscribed period of time from infancy to the onset of puberty.

Tolerance
Diminished or absent capacity to make a specific response to an antigen, usually produced as a result of contact with that antigen under nonimmunizing conditions.

Development
The process of growth and differentiation.



SIMILAR TERMS
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Fructans
Polysaccharides composed of D-fructose units.

Fructokinases
A class of enzymes that catalyzes the phosphorylation of fructose in the presence of ATP. EC 2.7.1.-.

Fructosamine
A term referring to the linking of blood sugar onto protein molecules in the bloodstream. The fructosamine value depends upon the average blood sugar level during the past three weeks. The fructosamine test could be viewed as complementary to the glycohemoglobin, as the two tests are different reflections of diabetes control: glycohemoglobin looks back approximately eight to twelve weeks, and the fructosamine test looks back about three weeks. |Note: the term fructosamine has nothing to do with the term fructose.

Fructose
Fruit sugar.

Fructose 1 Phosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose 1,6 Biphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose 1,6 Bisphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose 1,6 Bisphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose 1,6 Bisphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose 1,6 Diphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose 1,6 Diphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose 1,6-Bisphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose 1,6-Bisphosphate Aldolase, Class II
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose 1-Phosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose 2,6-bisphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose Biphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose Biphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose Bisphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose Bisphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose Intolerance
An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

Fructose Intolerances
An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

Fructose Metabolism, Inborn Errors
Inherited abnormalities of fructose metabolism, which include three known autosomal recessive types: hepatic fructokinase deficiency (essential fructosuria), hereditary fructose intolerance, and hereditary fructose-1,6-diphosphatase deficiency. Essential fructosuria is a benign asymptomatic metabolic disorder caused by deficiency in fructokinase, leading to decreased conversion of fructose to fructose-1-phosphate and alimentary hyperfructosemia, but with no clinical dysfunction; may produce a false-positive diabetes test.

Fructose-1,6-Biphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose-1,6-Bisphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose-1,6-Bisphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-1,6-Bisphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-1,6-Diphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose-1,6-Diphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-2,6-bisphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-2,6-bisphosphate 2-phosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-2,6-diphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-6-P 1-Kinase
An allosteric enzyme that regulates glycolysis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-1,6-bisphosphate. D-tagatose- 6-phosphate and sedoheptulose-7-phosphate also are acceptors. UTP, CTP, and ITP also are donors. In human phosphofructokinase-1, three types of subunits have been identified. They are muscle type M (PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE); liver type L (PHOSPHOFRUCTOKINASE-1, LIVER TYPE); and type C (PHOSPHOFRUCTOKINASE-1, C TYPE) found in platelets, brain, and other tissues.

Fructose-6-P,2-kinase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-6-phosphate 1-Phosphotransferase
An allosteric enzyme that regulates glycolysis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-1,6-bisphosphate. D-tagatose- 6-phosphate and sedoheptulose-7-phosphate also are acceptors. UTP, CTP, and ITP also are donors. In human phosphofructokinase-1, three types of subunits have been identified. They are muscle type M (PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE); liver type L (PHOSPHOFRUCTOKINASE-1, LIVER TYPE); and type C (PHOSPHOFRUCTOKINASE-1, C TYPE) found in platelets, brain, and other tissues.

Fructose-6-phosphate,2-kinase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-6-phosphate,2-kinase-fructose-2,6-bisphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-Biphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-Biphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-Bisphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose-Bisphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructosediphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructosediphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructosediphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructosediphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructosediphosphates
Diphosphoric acid esters of fructose. The fructose-1,6- diphosphate isomer is most prevalent. It is an important intermediate in the glycolysis process.

Fructosemonophosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructuronate Reductase
An enzyme that catalyzes the reversible oxidation of mannonate to fructuronate in the presence of NAD. Also reduces D-tagaturonate to D-altronate. EC 1.1.1.57.



PREVIOUS AND NEXT TERMS
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Fructose 1,6 Diphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-6-P,2-kinase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-2,6-diphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-2,6-bisphosphate 2-phosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-2,6-bisphosphatase
An allosteric enzyme that regulates glycolysis and gluconeogenesis by catalyzing the transfer of a phosphate group from ATP to fructose-6-phosphate to yield fructose-2,6-bisphosphate, an allosteric effector for the other 6-phosphofructokinase, PHOSPHOFRUCTOKINASE-1. Phosphofructokinase-2 is bifunctional: the dephosphorylated form is a kinase and the phosphorylated form is a phosphatase that breaks down fructose-2,6-bisphosphate to yield fructose-6-phosphate.

Fructose-1,6-Diphosphatase Deficiency

Fructose-1,6-Diphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-Bisphosphatase
An enzyme that catalyzes the conversion of D-fructose 1,6-bisphosphate and water to D-fructose 6-phosphate and orthophosphate. EC 3.1.3.11.

Fructose-Bisphosphate Aldolase
An enzyme of the lyase class that catalyzes the cleavage of fructose 1,6-biphosphate to form dihydroxyacetone phosphate and glyceraldehyde 3-phosphate. The enzyme also acts on (3S,4R)-ketose 1-phosphates. The yeast and bacterial enzymes are zinc proteins. (Enzyme Nomenclature, 1992) E.C. 4.1.2.13.

Fructose-Biphosphatase Deficiency
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

Fructose-Biphosphatase Deficiencies
An autosomal recessive fructose metabolism disorder due to absent or deficient fructose-1,6-diphosphatase activity. Gluconeogenesis is impaired, resulting in accumulation of gluconeogenic precursors (e.g., amino acids, lactate, ketones) and manifested as hypoglycemia, ketosis, and lactic acidosis. Episodes in the newborn infant are often lethal. Later episodes are often brought on by fasting and febrile infections. As patients age through early childhood, tolerance to fasting improves and development becomes normal.

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   fructose-1,6-diphosphatasedeficiency / ructose-1,6-diphosphatase deficiency / fuctose-1,6-diphosphatase deficiency / frctose-1,6-diphosphatase deficiency / frutose-1,6-diphosphatase deficiency / frucose-1,6-diphosphatase deficiency / fructse-1,6-diphosphatase deficiency / fructoe-1,6-diphosphatase deficiency / fructos-1,6-diphosphatase deficiency / fructose1,6-diphosphatase deficiency / fructose-,6-diphosphatase deficiency / fructose-16-diphosphatase deficiency / fructose-1,-diphosphatase deficiency / fructose-1,6diphosphatase deficiency / fructose-1,6-iphosphatase deficiency / fructose-1,6-dphosphatase deficiency / fructose-1,6-dihosphatase deficiency / fructose-1,6-diposphatase deficiency / fructose-1,6-diphsphatase deficiency / fructose-1,6-diphophatase deficiency / fructose-1,6-diphoshatase deficiency / fructose-1,6-diphospatase deficiency / fructose-1,6-diphosphtase deficiency / fructose-1,6-diphosphaase deficiency / fructose-1,6-diphosphatse deficiency / fructose-1,6-diphosphatae deficiency / fructose-1,6-diphosphatas deficiency / fructose-1,6-diphosphatasedeficiency / fructose-1,6-diphosphatase eficiency / fructose-1,6-diphosphatase dficiency / fructose-1,6-diphosphatase deiciency / fructose-1,6-diphosphatase defciency / fructose-1,6-diphosphatase defiiency / fructose-1,6-diphosphatase deficency / fructose-1,6-diphosphatase deficincy / fructose-1,6-diphosphatase deficiecy / fructose-1,6-diphosphatase deficieny / fructose-1,6-diphosphatase deficienc / ffructose-1,6-diphosphatase deficiency / frructose-1,6-diphosphatase deficiency / fruuctose-1,6-diphosphatase deficiency / frucctose-1,6-diphosphatase deficiency / fructtose-1,6-diphosphatase deficiency / fructoose-1,6-diphosphatase deficiency / fructosse-1,6-diphosphatase deficiency / fructosee-1,6-diphosphatase deficiency / fructose--1,6-diphosphatase deficiency / fructose-11,6-diphosphatase deficiency / fructose-1,,6-diphosphatase deficiency / fructose-1,66-diphosphatase deficiency / fructose-1,6--diphosphatase deficiency / fructose-1,6-ddiphosphatase deficiency / fructose-1,6-diiphosphatase deficiency / fructose-1,6-dipphosphatase deficiency / fructose-1,6-diphhosphatase deficiency / fructose-1,6-diphoosphatase deficiency / fructose-1,6-diphossphatase deficiency / fructose-1,6-diphospphatase deficiency / fructose-1,6-diphosphhatase deficiency / fructose-1,6-diphosphaatase deficiency / fructose-1,6-diphosphattase deficiency / fructose-1,6-diphosphataase deficiency / fructose-1,6-diphosphatasse deficiency / fructose-1,6-diphosphatasee deficiency / fructose-1,6-diphosphatase deficiency / fructose-1,6-diphosphatase ddeficiency / fructose-1,6-diphosphatase deeficiency / fructose-1,6-diphosphatase defficiency / fructose-1,6-diphosphatase defiiciency / fructose-1,6-diphosphatase deficciency / fructose-1,6-diphosphatase deficiiency / fructose-1,6-diphosphatase deficieency / fructose-1,6-diphosphatase deficienncy / fructose-1,6-diphosphatase deficienccy / fructose-1,6-diphosphatase deficiencyy / rructose-1,6-diphosphatase deficiency / tructose-1,6-diphosphatase deficiency / gructose-1,6-diphosphatase deficiency / bructose-1,6-diphosphatase deficiency / vructose-1,6-diphosphatase deficiency / cructose-1,6-diphosphatase deficiency / dructose-1,6-diphosphatase deficiency / eructose-1,6-diphosphatase deficiency / f4uctose-1,6-diphosphatase deficiency / f5uctose-1,6-diphosphatase deficiency / ftuctose-1,6-diphosphatase deficiency / fguctose-1,6-diphosphatase deficiency / ffuctose-1,6-diphosphatase deficiency / fductose-1,6-diphosphatase deficiency / feuctose-1,6-diphosphatase deficiency / f3uctose-1,6-diphosphatase deficiency / fr7ctose-1,6-diphosphatase deficiency / fr8ctose-1,6-diphosphatase deficiency / frictose-1,6-diphosphatase deficiency / frkctose-1,6-diphosphatase deficiency / frjctose-1,6-diphosphatase deficiency / frhctose-1,6-diphosphatase deficiency / fryctose-1,6-diphosphatase deficiency / fr6ctose-1,6-diphosphatase deficiency / fruxtose-1,6-diphosphatase deficiency / frustose-1,6-diphosphatase deficiency / frudtose-1,6-diphosphatase deficiency / fruftose-1,6-diphosphatase deficiency / fruvtose-1,6-diphosphatase deficiency / fru tose-1,6-diphosphatase deficiency / fruc5ose-1,6-diphosphatase deficiency / fruc6ose-1,6-diphosphatase deficiency / frucyose-1,6-diphosphatase deficiency / fruchose-1,6-diphosphatase deficiency / frucgose-1,6-diphosphatase deficiency / frucfose-1,6-diphosphatase deficiency / frucrose-1,6-diphosphatase deficiency / fruc4ose-1,6-diphosphatase deficiency / fruct9se-1,6-diphosphatase deficiency / fruct0se-1,6-diphosphatase deficiency / fructpse-1,6-diphosphatase deficiency / fructlse-1,6-diphosphatase deficiency / fructkse-1,6-diphosphatase deficiency / fructise-1,6-diphosphatase deficiency / fruct8se-1,6-diphosphatase deficiency / fructowe-1,6-diphosphatase deficiency / fructoee-1,6-diphosphatase deficiency / fructode-1,6-diphosphatase deficiency / fructoxe-1,6-diphosphatase deficiency / fructoze-1,6-diphosphatase deficiency / fructoae-1,6-diphosphatase deficiency / fructoqe-1,6-diphosphatase deficiency / fructos3-1,6-diphosphatase deficiency / fructos4-1,6-diphosphatase deficiency / fructosr-1,6-diphosphatase deficiency / fructosf-1,6-diphosphatase deficiency / fructosd-1,6-diphosphatase deficiency / fructoss-1,6-diphosphatase deficiency / fructosw-1,6-diphosphatase deficiency / fructose=1,6-diphosphatase deficiency / fructose[1,6-diphosphatase deficiency / fructosep1,6-diphosphatase deficiency / fructose01,6-diphosphatase deficiency / fructose-2,6-diphosphatase deficiency / fructose-w,6-diphosphatase deficiency / fructose-q,6-diphosphatase deficiency / fructose-`,6-diphosphatase deficiency / fructose-1,7-diphosphatase deficiency / fructose-1,u-diphosphatase deficiency / fructose-1,y-diphosphatase deficiency / fructose-1,t-diphosphatase deficiency / fructose-1,5-diphosphatase deficiency / fructose-1,6=diphosphatase deficiency / fructose-1,6[diphosphatase deficiency / fructose-1,6pdiphosphatase deficiency / fructose-1,60diphosphatase deficiency / fructose-1,6-eiphosphatase deficiency / fructose-1,6-riphosphatase deficiency / fructose-1,6-fiphosphatase deficiency / fructose-1,6-viphosphatase deficiency / fructose-1,6-ciphosphatase deficiency / fructose-1,6-xiphosphatase deficiency / fructose-1,6-siphosphatase deficiency / fructose-1,6-wiphosphatase deficiency / fructose-1,6-dphosphatase deficiency / fructose-1,6-di0hosphatase deficiency / fructose-1,6-di-hosphatase deficiency / fructose-1,6-di[hosphatase deficiency / fructose-1,6-di;hosphatase deficiency / fructose-1,6-dilhosphatase deficiency / fructose-1,6-diohosphatase deficiency / fructose-1,6-di9hosphatase deficiency / fructose-1,6-dipyosphatase deficiency / fructose-1,6-dipuosphatase deficiency / fructose-1,6-dipjosphatase deficiency / fructose-1,6-dipnosphatase deficiency / fructose-1,6-dipbosphatase deficiency / fructose-1,6-dipgosphatase deficiency / fructose-1,6-diptosphatase deficiency / fructose-1,6-diph9sphatase deficiency / fructose-1,6-diph0sphatase deficiency / fructose-1,6-diphpsphatase deficiency / fructose-1,6-diphlsphatase deficiency / fructose-1,6-diphksphatase deficiency / fructose-1,6-diphisphatase deficiency / fructose-1,6-diph8sphatase deficiency / fructose-1,6-diphowphatase deficiency / fructose-1,6-diphoephatase deficiency / fructose-1,6-diphodphatase deficiency / fructose-1,6-diphoxphatase deficiency / fructose-1,6-diphozphatase deficiency / fructose-1,6-diphoaphatase deficiency / fructose-1,6-diphoqphatase deficiency / fructose-1,6-diphos0hatase deficiency / fructose-1,6-diphos-hatase deficiency / fructose-1,6-diphos[hatase deficiency / fructose-1,6-diphos;hatase deficiency / fructose-1,6-diphoslhatase deficiency / fructose-1,6-diphosohatase deficiency / fructose-1,6-diphos9hatase deficiency / fructose-1,6-diphospyatase deficiency / fructose-1,6-diphospuatase deficiency / fructose-1,6-diphospjatase deficiency / fructose-1,6-diphospnatase deficiency / fructose-1,6-diphospbatase deficiency / fructose-1,6-diphospgatase deficiency / fructose-1,6-diphosptatase deficiency / fructose-1,6-diphosphqtase deficiency / fructose-1,6-diphosphwtase deficiency / fructose-1,6-diphosphstase deficiency / fructose-1,6-diphosphxtase deficiency / fructose-1,6-diphosphztase deficiency / fructose-1,6-diphospha5ase deficiency / fructose-1,6-diphospha6ase deficiency / fructose-1,6-diphosphayase deficiency / fructose-1,6-diphosphahase deficiency / fructose-1,6-diphosphagase deficiency / fructose-1,6-diphosphafase deficiency / fructose-1,6-diphospharase deficiency / fructose-1,6-diphospha4ase deficiency / fructose-1,6-diphosphatqse deficiency / fructose-1,6-diphosphatwse deficiency / fructose-1,6-diphosphatsse deficiency / fructose-1,6-diphosphatxse deficiency / fructose-1,6-diphosphatzse deficiency / fructose-1,6-diphosphatawe deficiency / fructose-1,6-diphosphataee deficiency / fructose-1,6-diphosphatade deficiency / fructose-1,6-diphosphataxe deficiency / fructose-1,6-diphosphataze deficiency / fructose-1,6-diphosphataae deficiency / fructose-1,6-diphosphataqe deficiency / fructose-1,6-diphosphatas3 deficiency / fructose-1,6-diphosphatas4 deficiency / fructose-1,6-diphosphatasr deficiency / fructose-1,6-diphosphatasf deficiency / fructose-1,6-diphosphatasd deficiency / fructose-1,6-diphosphatass deficiency / fructose-1,6-diphosphatasw deficiency / fructose-1,6-diphosphatase eeficiency / fructose-1,6-diphosphatase reficiency / fructose-1,6-diphosphatase feficiency / fructose-1,6-diphosphatase veficiency / fructose-1,6-diphosphatase ceficiency / fructose-1,6-diphosphatase xeficiency / fructose-1,6-diphosphatase seficiency / fructose-1,6-diphosphatase weficiency / fructose-1,6-diphosphatase d3ficiency / fructose-1,6-diphosphatase d4ficiency / fructose-1,6-diphosphatase drficiency / fructose-1,6-diphosphatase dfficiency / fructose-1,6-diphosphatase ddficiency / fructose-1,6-diphosphatase dsficiency / fructose-1,6-diphosphatase dwficiency / fructose-1,6-diphosphatase dericiency / fructose-1,6-diphosphatase deticiency / fructose-1,6-diphosphatase degiciency / fructose-1,6-diphosphatase debiciency / fructose-1,6-diphosphatase deviciency / fructose-1,6-diphosphatase deciciency / fructose-1,6-diphosphatase dediciency / fructose-1,6-diphosphatase deeiciency / fructose-1,6-diphosphatase defciency / fructose-1,6-diphosphatase defixiency / fructose-1,6-diphosphatase defisiency / fructose-1,6-diphosphatase defidiency / fructose-1,6-diphosphatase defifiency / fructose-1,6-diphosphatase defiviency / fructose-1,6-diphosphatase defi iency / fructose-1,6-diphosphatase deficency / fructose-1,6-diphosphatase defici3ncy / fructose-1,6-diphosphatase defici4ncy / fructose-1,6-diphosphatase deficirncy / fructose-1,6-diphosphatase deficifncy / fructose-1,6-diphosphatase deficidncy / fructose-1,6-diphosphatase deficisncy / fructose-1,6-diphosphatase deficiwncy / fructose-1,6-diphosphatase deficiebcy / fructose-1,6-diphosphatase deficiehcy / fructose-1,6-diphosphatase deficiejcy / fructose-1,6-diphosphatase deficiemcy / fructose-1,6-diphosphatase deficie cy / fructose-1,6-diphosphatase deficienxy / fructose-1,6-diphosphatase deficiensy / fructose-1,6-diphosphatase deficiendy / fructose-1,6-diphosphatase deficienfy / fructose-1,6-diphosphatase deficienvy / fructose-1,6-diphosphatase deficien y / fructose-1,6-diphosphatase deficienc6 / fructose-1,6-diphosphatase deficienc7 / fructose-1,6-diphosphatase deficiencu / fructose-1,6-diphosphatase deficiencj / fructose-1,6-diphosphatase deficiench / fructose-1,6-diphosphatase deficiencg / fructose-1,6-diphosphatase deficienct / fructose-1,6-diphosphatase deficienc5 /