Fructose 1,6 Bisphosphatase Deficiency
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   Fructose 1,6 Bisphosphatase Deficiency


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.

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 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-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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Fronto-Polar Epilepsy, Anterior
A localization-related (focal) form of epilepsy characterized by seizures which arise in the frontal lobe. A variety of clinical syndromes exist depending on the exact location of the seizure focus. Simple or complex motor movements may occur, and most commonly involve the face and upper extremities. Seizures in the anterior frontal regions may be associated with head and eye turning, typically away from the side of origin of the seizure. Frontal lobe seizures may be idiopathic (cryptogenic) or caused by an identifiable disease process such as traumatic injuries, neoplasms, or other macroscopic or microscopic lesions of the frontal lobes (symptomatic frontal lobe seizures). (From Adams et al., Principles of Neurology, 6th ed, pp318-9)

Fronto-Polar Epilepsies, Anterior
A localization-related (focal) form of epilepsy characterized by seizures which arise in the frontal lobe. A variety of clinical syndromes exist depending on the exact location of the seizure focus. Simple or complex motor movements may occur, and most commonly involve the face and upper extremities. Seizures in the anterior frontal regions may be associated with head and eye turning, typically away from the side of origin of the seizure. Frontal lobe seizures may be idiopathic (cryptogenic) or caused by an identifiable disease process such as traumatic injuries, neoplasms, or other macroscopic or microscopic lesions of the frontal lobes (symptomatic frontal lobe seizures). (From Adams et al., Principles of Neurology, 6th ed, pp318-9)

Frontal Sinusitis
Inflammation of the frontal sinus; in most cases the infection is caused by the bacteria STREPTOCOCCUS PNEUMONIAE and HAEMOPHILUS INFLUENZAE. This condition may be acute or chronic.

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 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

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 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.

Fru-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.

FRP-1 protein
A heterodimeric protein that is a cell surface antigen associated with lymphocyte activation. The initial characterization of this protein revealed one identifiable heavy chain (CD98 HEAVY CHAIN) and an indeterminate smaller light chain. It is now known that a variety of light chain subunits (CD98 LIGHT CHAINS) can dimerize with the heavy chain. Depending upon its light chain composition a diverse array of functions can be found for this protein. Functions include: type L amino acid transport, type y+L amino acid transport and regulation of cellular fusion.

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.

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bisphosphatase deficiency / frhctose 1,6 bisphosphatase deficiency / fryctose 1,6 bisphosphatase deficiency / fr6ctose 1,6 bisphosphatase deficiency / fruxtose 1,6 bisphosphatase deficiency / frustose 1,6 bisphosphatase deficiency / frudtose 1,6 bisphosphatase deficiency / fruftose 1,6 bisphosphatase deficiency / fruvtose 1,6 bisphosphatase deficiency / fru tose 1,6 bisphosphatase deficiency / fruc5ose 1,6 bisphosphatase deficiency / fruc6ose 1,6 bisphosphatase deficiency / frucyose 1,6 bisphosphatase deficiency / fruchose 1,6 bisphosphatase deficiency / frucgose 1,6 bisphosphatase deficiency / frucfose 1,6 bisphosphatase deficiency / frucrose 1,6 bisphosphatase deficiency / fruc4ose 1,6 bisphosphatase deficiency / fruct9se 1,6 bisphosphatase deficiency / fruct0se 1,6 bisphosphatase deficiency / fructpse 1,6 bisphosphatase deficiency / fructlse 1,6 bisphosphatase deficiency / fructkse 1,6 bisphosphatase deficiency / fructise 1,6 bisphosphatase deficiency / fruct8se 1,6 bisphosphatase deficiency / fructowe 1,6 bisphosphatase deficiency / fructoee 1,6 bisphosphatase deficiency / fructode 1,6 bisphosphatase deficiency / fructoxe 1,6 bisphosphatase deficiency / fructoze 1,6 bisphosphatase deficiency / fructoae 1,6 bisphosphatase deficiency / fructoqe 1,6 bisphosphatase deficiency / fructos3 1,6 bisphosphatase deficiency / fructos4 1,6 bisphosphatase deficiency / fructosr 1,6 bisphosphatase deficiency / fructosf 1,6 bisphosphatase deficiency / fructosd 1,6 bisphosphatase deficiency / fructoss 1,6 bisphosphatase deficiency / fructosw 1,6 bisphosphatase deficiency / fructose 2,6 bisphosphatase deficiency / fructose w,6 bisphosphatase deficiency / fructose q,6 bisphosphatase deficiency / fructose `,6 bisphosphatase deficiency / fructose 1,7 bisphosphatase deficiency / fructose 1,u bisphosphatase deficiency / fructose 1,y bisphosphatase deficiency / fructose 1,t bisphosphatase deficiency / fructose 1,5 bisphosphatase deficiency / fructose 1,6 visphosphatase deficiency / fructose 1,6 fisphosphatase deficiency / fructose 1,6 gisphosphatase deficiency / fructose 1,6 hisphosphatase deficiency / fructose 1,6 nisphosphatase deficiency / fructose 1,6 isphosphatase deficiency / fructose 1,6 bsphosphatase deficiency / fructose 1,6 biwphosphatase deficiency / fructose 1,6 biephosphatase deficiency / fructose 1,6 bidphosphatase deficiency / fructose 1,6 bixphosphatase deficiency / fructose 1,6 bizphosphatase deficiency / fructose 1,6 biaphosphatase deficiency / fructose 1,6 biqphosphatase deficiency / fructose 1,6 bis0hosphatase deficiency / fructose 1,6 bis-hosphatase deficiency / fructose 1,6 bis[hosphatase deficiency / fructose 1,6 bis;hosphatase deficiency / fructose 1,6 bislhosphatase deficiency / fructose 1,6 bisohosphatase deficiency / fructose 1,6 bis9hosphatase deficiency / fructose 1,6 bispyosphatase deficiency / fructose 1,6 bispuosphatase deficiency / fructose 1,6 bispjosphatase deficiency / fructose 1,6 bispnosphatase deficiency / fructose 1,6 bispbosphatase deficiency / fructose 1,6 bispgosphatase deficiency / fructose 1,6 bisptosphatase deficiency / fructose 1,6 bisph9sphatase deficiency / fructose 1,6 bisph0sphatase deficiency / fructose 1,6 bisphpsphatase deficiency / fructose 1,6 bisphlsphatase deficiency / fructose 1,6 bisphksphatase deficiency / fructose 1,6 bisphisphatase deficiency / fructose 1,6 bisph8sphatase deficiency / fructose 1,6 bisphowphatase deficiency / fructose 1,6 bisphoephatase deficiency / fructose 1,6 bisphodphatase deficiency / fructose 1,6 bisphoxphatase deficiency / fructose 1,6 bisphozphatase deficiency / fructose 1,6 bisphoaphatase deficiency / fructose 1,6 bisphoqphatase deficiency / fructose 1,6 bisphos0hatase deficiency / fructose 1,6 bisphos-hatase deficiency / fructose 1,6 bisphos[hatase deficiency / fructose 1,6 bisphos;hatase deficiency / fructose 1,6 bisphoslhatase deficiency / fructose 1,6 bisphosohatase deficiency / fructose 1,6 bisphos9hatase deficiency / fructose 1,6 bisphospyatase deficiency / fructose 1,6 bisphospuatase deficiency / fructose 1,6 bisphospjatase deficiency / fructose 1,6 bisphospnatase deficiency / fructose 1,6 bisphospbatase deficiency / fructose 1,6 bisphospgatase deficiency / fructose 1,6 bisphosptatase deficiency / fructose 1,6 bisphosphqtase deficiency / fructose 1,6 bisphosphwtase deficiency / fructose 1,6 bisphosphstase deficiency / fructose 1,6 bisphosphxtase deficiency / fructose 1,6 bisphosphztase deficiency / fructose 1,6 bisphospha5ase deficiency / fructose 1,6 bisphospha6ase deficiency / fructose 1,6 bisphosphayase deficiency / fructose 1,6 bisphosphahase deficiency / fructose 1,6 bisphosphagase deficiency / fructose 1,6 bisphosphafase deficiency / fructose 1,6 bisphospharase deficiency / fructose 1,6 bisphospha4ase deficiency / fructose 1,6 bisphosphatqse deficiency / fructose 1,6 bisphosphatwse deficiency / fructose 1,6 bisphosphatsse deficiency / fructose 1,6 bisphosphatxse deficiency / fructose 1,6 bisphosphatzse deficiency / fructose 1,6 bisphosphatawe deficiency / fructose 1,6 bisphosphataee deficiency / fructose 1,6 bisphosphatade deficiency / fructose 1,6 bisphosphataxe deficiency / fructose 1,6 bisphosphataze deficiency / fructose 1,6 bisphosphataae deficiency / fructose 1,6 bisphosphataqe deficiency / fructose 1,6 bisphosphatas3 deficiency / fructose 1,6 bisphosphatas4 deficiency / fructose 1,6 bisphosphatasr deficiency / fructose 1,6 bisphosphatasf deficiency / fructose 1,6 bisphosphatasd deficiency / fructose 1,6 bisphosphatass deficiency / fructose 1,6 bisphosphatasw deficiency / fructose 1,6 bisphosphatase eeficiency / fructose 1,6 bisphosphatase reficiency / fructose 1,6 bisphosphatase feficiency / fructose 1,6 bisphosphatase veficiency / fructose 1,6 bisphosphatase ceficiency / fructose 1,6 bisphosphatase xeficiency / fructose 1,6 bisphosphatase seficiency / fructose 1,6 bisphosphatase weficiency / fructose 1,6 bisphosphatase d3ficiency / fructose 1,6 bisphosphatase d4ficiency / fructose 1,6 bisphosphatase drficiency / fructose 1,6 bisphosphatase dfficiency / fructose 1,6 bisphosphatase ddficiency / fructose 1,6 bisphosphatase dsficiency / fructose 1,6 bisphosphatase dwficiency / fructose 1,6 bisphosphatase dericiency / fructose 1,6 bisphosphatase deticiency / fructose 1,6 bisphosphatase degiciency / fructose 1,6 bisphosphatase debiciency / fructose 1,6 bisphosphatase deviciency / fructose 1,6 bisphosphatase deciciency / fructose 1,6 bisphosphatase dediciency / fructose 1,6 bisphosphatase deeiciency / fructose 1,6 bisphosphatase defciency / fructose 1,6 bisphosphatase defixiency / fructose 1,6 bisphosphatase defisiency / fructose 1,6 bisphosphatase defidiency / fructose 1,6 bisphosphatase defifiency / fructose 1,6 bisphosphatase defiviency / fructose 1,6 bisphosphatase defi iency / fructose 1,6 bisphosphatase deficency / fructose 1,6 bisphosphatase defici3ncy / fructose 1,6 bisphosphatase defici4ncy / fructose 1,6 bisphosphatase deficirncy / fructose 1,6 bisphosphatase deficifncy / fructose 1,6 bisphosphatase deficidncy / fructose 1,6 bisphosphatase deficisncy / fructose 1,6 bisphosphatase deficiwncy / fructose 1,6 bisphosphatase deficiebcy / fructose 1,6 bisphosphatase deficiehcy / fructose 1,6 bisphosphatase deficiejcy / fructose 1,6 bisphosphatase deficiemcy / fructose 1,6 bisphosphatase deficie cy / fructose 1,6 bisphosphatase deficienxy / fructose 1,6 bisphosphatase deficiensy / fructose 1,6 bisphosphatase deficiendy / fructose 1,6 bisphosphatase deficienfy / fructose 1,6 bisphosphatase deficienvy / fructose 1,6 bisphosphatase deficien y / fructose 1,6 bisphosphatase deficienc6 / fructose 1,6 bisphosphatase deficienc7 / fructose 1,6 bisphosphatase deficiencu / fructose 1,6 bisphosphatase deficiencj / fructose 1,6 bisphosphatase deficiench / fructose 1,6 bisphosphatase deficiencg / fructose 1,6 bisphosphatase deficienct / fructose 1,6 bisphosphatase deficienc5 /