Fructose-1,6-Diphosphatase Deficiencies
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  Fructose-1,6-Diphosphatase Deficiencies



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.

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 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-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-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
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 Deficiencies

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.

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.

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fructose--1,6-diphosphatase deficiencies / fructose-11,6-diphosphatase deficiencies / fructose-1,,6-diphosphatase deficiencies / fructose-1,66-diphosphatase deficiencies / fructose-1,6--diphosphatase deficiencies / fructose-1,6-ddiphosphatase deficiencies / fructose-1,6-diiphosphatase deficiencies / fructose-1,6-dipphosphatase deficiencies / fructose-1,6-diphhosphatase deficiencies / fructose-1,6-diphoosphatase deficiencies / fructose-1,6-diphossphatase deficiencies / fructose-1,6-diphospphatase deficiencies / fructose-1,6-diphosphhatase deficiencies / fructose-1,6-diphosphaatase deficiencies / fructose-1,6-diphosphattase deficiencies / fructose-1,6-diphosphataase deficiencies / fructose-1,6-diphosphatasse deficiencies / fructose-1,6-diphosphatasee deficiencies / fructose-1,6-diphosphatase deficiencies / fructose-1,6-diphosphatase ddeficiencies / fructose-1,6-diphosphatase deeficiencies / fructose-1,6-diphosphatase defficiencies / fructose-1,6-diphosphatase defiiciencies / fructose-1,6-diphosphatase deficciencies / fructose-1,6-diphosphatase deficiiencies / fructose-1,6-diphosphatase deficieencies / fructose-1,6-diphosphatase deficienncies / fructose-1,6-diphosphatase deficienccies / fructose-1,6-diphosphatase deficienciies / fructose-1,6-diphosphatase deficienciees / fructose-1,6-diphosphatase deficienciess / rructose-1,6-diphosphatase deficiencies / tructose-1,6-diphosphatase deficiencies / gructose-1,6-diphosphatase deficiencies / bructose-1,6-diphosphatase deficiencies / vructose-1,6-diphosphatase deficiencies / cructose-1,6-diphosphatase deficiencies / dructose-1,6-diphosphatase deficiencies / eructose-1,6-diphosphatase deficiencies / f4uctose-1,6-diphosphatase deficiencies / f5uctose-1,6-diphosphatase deficiencies / ftuctose-1,6-diphosphatase deficiencies / fguctose-1,6-diphosphatase deficiencies / ffuctose-1,6-diphosphatase deficiencies / fductose-1,6-diphosphatase deficiencies / feuctose-1,6-diphosphatase deficiencies / f3uctose-1,6-diphosphatase deficiencies / fr7ctose-1,6-diphosphatase deficiencies / fr8ctose-1,6-diphosphatase deficiencies / frictose-1,6-diphosphatase deficiencies / frkctose-1,6-diphosphatase deficiencies / frjctose-1,6-diphosphatase deficiencies / frhctose-1,6-diphosphatase deficiencies / fryctose-1,6-diphosphatase deficiencies / fr6ctose-1,6-diphosphatase deficiencies / fruxtose-1,6-diphosphatase deficiencies / frustose-1,6-diphosphatase deficiencies / frudtose-1,6-diphosphatase deficiencies / fruftose-1,6-diphosphatase deficiencies / fruvtose-1,6-diphosphatase deficiencies / fru tose-1,6-diphosphatase deficiencies / fruc5ose-1,6-diphosphatase deficiencies / fruc6ose-1,6-diphosphatase deficiencies / frucyose-1,6-diphosphatase deficiencies / fruchose-1,6-diphosphatase deficiencies / frucgose-1,6-diphosphatase deficiencies / frucfose-1,6-diphosphatase deficiencies / frucrose-1,6-diphosphatase deficiencies / fruc4ose-1,6-diphosphatase deficiencies / fruct9se-1,6-diphosphatase deficiencies / fruct0se-1,6-diphosphatase deficiencies / fructpse-1,6-diphosphatase deficiencies / fructlse-1,6-diphosphatase deficiencies / fructkse-1,6-diphosphatase deficiencies / fructise-1,6-diphosphatase deficiencies / fruct8se-1,6-diphosphatase deficiencies / fructowe-1,6-diphosphatase deficiencies / fructoee-1,6-diphosphatase deficiencies / fructode-1,6-diphosphatase deficiencies / fructoxe-1,6-diphosphatase deficiencies / fructoze-1,6-diphosphatase deficiencies / fructoae-1,6-diphosphatase deficiencies / fructoqe-1,6-diphosphatase deficiencies / fructos3-1,6-diphosphatase deficiencies / fructos4-1,6-diphosphatase deficiencies / fructosr-1,6-diphosphatase deficiencies / fructosf-1,6-diphosphatase deficiencies / fructosd-1,6-diphosphatase deficiencies / fructoss-1,6-diphosphatase deficiencies / fructosw-1,6-diphosphatase deficiencies / fructose=1,6-diphosphatase deficiencies / fructose[1,6-diphosphatase deficiencies / fructosep1,6-diphosphatase deficiencies / fructose01,6-diphosphatase deficiencies / fructose-2,6-diphosphatase deficiencies / fructose-w,6-diphosphatase deficiencies / fructose-q,6-diphosphatase deficiencies / fructose-`,6-diphosphatase deficiencies / fructose-1,7-diphosphatase deficiencies / fructose-1,u-diphosphatase deficiencies / fructose-1,y-diphosphatase deficiencies / fructose-1,t-diphosphatase deficiencies / fructose-1,5-diphosphatase deficiencies / fructose-1,6=diphosphatase deficiencies / fructose-1,6[diphosphatase deficiencies / fructose-1,6pdiphosphatase deficiencies / fructose-1,60diphosphatase deficiencies / fructose-1,6-eiphosphatase deficiencies / fructose-1,6-riphosphatase deficiencies / fructose-1,6-fiphosphatase deficiencies / fructose-1,6-viphosphatase deficiencies / fructose-1,6-ciphosphatase deficiencies / fructose-1,6-xiphosphatase deficiencies / fructose-1,6-siphosphatase deficiencies / fructose-1,6-wiphosphatase deficiencies / fructose-1,6-dphosphatase deficiencies / fructose-1,6-di0hosphatase deficiencies / fructose-1,6-di-hosphatase deficiencies / fructose-1,6-di[hosphatase deficiencies / fructose-1,6-di;hosphatase deficiencies / fructose-1,6-dilhosphatase deficiencies / fructose-1,6-diohosphatase deficiencies / fructose-1,6-di9hosphatase deficiencies / fructose-1,6-dipyosphatase deficiencies / fructose-1,6-dipuosphatase deficiencies / fructose-1,6-dipjosphatase deficiencies / fructose-1,6-dipnosphatase deficiencies / fructose-1,6-dipbosphatase deficiencies / fructose-1,6-dipgosphatase deficiencies / fructose-1,6-diptosphatase deficiencies / fructose-1,6-diph9sphatase deficiencies / fructose-1,6-diph0sphatase deficiencies / fructose-1,6-diphpsphatase deficiencies / fructose-1,6-diphlsphatase deficiencies / fructose-1,6-diphksphatase deficiencies / fructose-1,6-diphisphatase deficiencies / fructose-1,6-diph8sphatase deficiencies / fructose-1,6-diphowphatase deficiencies / fructose-1,6-diphoephatase deficiencies / fructose-1,6-diphodphatase deficiencies / fructose-1,6-diphoxphatase deficiencies / fructose-1,6-diphozphatase deficiencies / fructose-1,6-diphoaphatase deficiencies / fructose-1,6-diphoqphatase deficiencies / fructose-1,6-diphos0hatase deficiencies / fructose-1,6-diphos-hatase deficiencies / fructose-1,6-diphos[hatase deficiencies / fructose-1,6-diphos;hatase deficiencies / fructose-1,6-diphoslhatase deficiencies / fructose-1,6-diphosohatase deficiencies / fructose-1,6-diphos9hatase deficiencies / fructose-1,6-diphospyatase deficiencies / fructose-1,6-diphospuatase deficiencies / fructose-1,6-diphospjatase deficiencies / fructose-1,6-diphospnatase deficiencies / fructose-1,6-diphospbatase deficiencies / fructose-1,6-diphospgatase deficiencies / fructose-1,6-diphosptatase deficiencies / fructose-1,6-diphosphqtase deficiencies / fructose-1,6-diphosphwtase deficiencies / fructose-1,6-diphosphstase deficiencies / fructose-1,6-diphosphxtase deficiencies / fructose-1,6-diphosphztase deficiencies / fructose-1,6-diphospha5ase deficiencies / fructose-1,6-diphospha6ase deficiencies / fructose-1,6-diphosphayase deficiencies / fructose-1,6-diphosphahase deficiencies / fructose-1,6-diphosphagase deficiencies / fructose-1,6-diphosphafase deficiencies / fructose-1,6-diphospharase deficiencies / fructose-1,6-diphospha4ase deficiencies / fructose-1,6-diphosphatqse deficiencies / fructose-1,6-diphosphatwse deficiencies / fructose-1,6-diphosphatsse deficiencies / fructose-1,6-diphosphatxse deficiencies / fructose-1,6-diphosphatzse deficiencies / fructose-1,6-diphosphatawe deficiencies / fructose-1,6-diphosphataee deficiencies / fructose-1,6-diphosphatade deficiencies / fructose-1,6-diphosphataxe deficiencies / fructose-1,6-diphosphataze deficiencies / fructose-1,6-diphosphataae deficiencies / fructose-1,6-diphosphataqe deficiencies / fructose-1,6-diphosphatas3 deficiencies / fructose-1,6-diphosphatas4 deficiencies / fructose-1,6-diphosphatasr deficiencies / fructose-1,6-diphosphatasf deficiencies / fructose-1,6-diphosphatasd deficiencies / fructose-1,6-diphosphatass deficiencies / fructose-1,6-diphosphatasw deficiencies / fructose-1,6-diphosphatase eeficiencies / fructose-1,6-diphosphatase reficiencies / fructose-1,6-diphosphatase feficiencies / fructose-1,6-diphosphatase veficiencies / fructose-1,6-diphosphatase ceficiencies / fructose-1,6-diphosphatase xeficiencies / fructose-1,6-diphosphatase seficiencies / fructose-1,6-diphosphatase weficiencies / fructose-1,6-diphosphatase d3ficiencies / fructose-1,6-diphosphatase d4ficiencies / fructose-1,6-diphosphatase drficiencies / fructose-1,6-diphosphatase dfficiencies / fructose-1,6-diphosphatase ddficiencies / fructose-1,6-diphosphatase dsficiencies / fructose-1,6-diphosphatase dwficiencies / fructose-1,6-diphosphatase dericiencies / fructose-1,6-diphosphatase deticiencies / fructose-1,6-diphosphatase degiciencies / fructose-1,6-diphosphatase debiciencies / fructose-1,6-diphosphatase deviciencies / fructose-1,6-diphosphatase deciciencies / fructose-1,6-diphosphatase dediciencies / fructose-1,6-diphosphatase deeiciencies / fructose-1,6-diphosphatase defciencies / fructose-1,6-diphosphatase defixiencies / fructose-1,6-diphosphatase defisiencies / fructose-1,6-diphosphatase defidiencies / fructose-1,6-diphosphatase defifiencies / fructose-1,6-diphosphatase defiviencies / fructose-1,6-diphosphatase defi iencies / fructose-1,6-diphosphatase deficencies / fructose-1,6-diphosphatase defici3ncies / fructose-1,6-diphosphatase defici4ncies / fructose-1,6-diphosphatase deficirncies / fructose-1,6-diphosphatase deficifncies / fructose-1,6-diphosphatase deficidncies / fructose-1,6-diphosphatase deficisncies / fructose-1,6-diphosphatase deficiwncies / fructose-1,6-diphosphatase deficiebcies / fructose-1,6-diphosphatase deficiehcies / fructose-1,6-diphosphatase deficiejcies / fructose-1,6-diphosphatase deficiemcies / fructose-1,6-diphosphatase deficie cies / fructose-1,6-diphosphatase deficienxies / fructose-1,6-diphosphatase deficiensies / fructose-1,6-diphosphatase deficiendies / fructose-1,6-diphosphatase deficienfies / fructose-1,6-diphosphatase deficienvies / fructose-1,6-diphosphatase deficien ies / fructose-1,6-diphosphatase deficiences / fructose-1,6-diphosphatase deficienci3s / fructose-1,6-diphosphatase deficienci4s / fructose-1,6-diphosphatase deficiencirs / fructose-1,6-diphosphatase deficiencifs / fructose-1,6-diphosphatase deficiencids / fructose-1,6-diphosphatase deficienciss / fructose-1,6-diphosphatase deficienciws / fructose-1,6-diphosphatase deficienciew / fructose-1,6-diphosphatase deficienciee / fructose-1,6-diphosphatase deficiencied / fructose-1,6-diphosphatase deficienciex / fructose-1,6-diphosphatase deficienciez / fructose-1,6-diphosphatase deficienciea / fructose-1,6-diphosphatase deficiencieq /