Fructose-1,6-Bisphosphatase Deficiencies
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  Fructose-1,6-Bisphosphatase Deficiencies



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.

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 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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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
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-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-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-1,6-Bisphosphatase Deficiencies

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-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-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.

FRP-1 Heavy Chain
A transmembrane glycoprotein subunit that can dimerize with a variety of light chain subunits (ANTIGENS CD98, LIGHT CHAINS). This protein subunit serves a diverse array of functions including amino acid transport and cell fusion. Its function is altered depending which of the light chain subunits it interacts with.

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ddeficiencies / fructose-1,6-bisphosphatase deeficiencies / fructose-1,6-bisphosphatase defficiencies / fructose-1,6-bisphosphatase defiiciencies / fructose-1,6-bisphosphatase deficciencies / fructose-1,6-bisphosphatase deficiiencies / fructose-1,6-bisphosphatase deficieencies / fructose-1,6-bisphosphatase deficienncies / fructose-1,6-bisphosphatase deficienccies / fructose-1,6-bisphosphatase deficienciies / fructose-1,6-bisphosphatase deficienciees / fructose-1,6-bisphosphatase deficienciess / rructose-1,6-bisphosphatase deficiencies / tructose-1,6-bisphosphatase deficiencies / gructose-1,6-bisphosphatase deficiencies / bructose-1,6-bisphosphatase deficiencies / vructose-1,6-bisphosphatase deficiencies / cructose-1,6-bisphosphatase deficiencies / dructose-1,6-bisphosphatase deficiencies / eructose-1,6-bisphosphatase deficiencies / f4uctose-1,6-bisphosphatase deficiencies / f5uctose-1,6-bisphosphatase deficiencies / ftuctose-1,6-bisphosphatase deficiencies / fguctose-1,6-bisphosphatase deficiencies / ffuctose-1,6-bisphosphatase deficiencies / fductose-1,6-bisphosphatase deficiencies / feuctose-1,6-bisphosphatase deficiencies / f3uctose-1,6-bisphosphatase deficiencies / fr7ctose-1,6-bisphosphatase deficiencies / fr8ctose-1,6-bisphosphatase deficiencies / frictose-1,6-bisphosphatase deficiencies / frkctose-1,6-bisphosphatase deficiencies / frjctose-1,6-bisphosphatase deficiencies / frhctose-1,6-bisphosphatase deficiencies / fryctose-1,6-bisphosphatase deficiencies / fr6ctose-1,6-bisphosphatase deficiencies / fruxtose-1,6-bisphosphatase deficiencies / frustose-1,6-bisphosphatase deficiencies / frudtose-1,6-bisphosphatase deficiencies / fruftose-1,6-bisphosphatase deficiencies / fruvtose-1,6-bisphosphatase deficiencies / fru tose-1,6-bisphosphatase deficiencies / fruc5ose-1,6-bisphosphatase deficiencies / fruc6ose-1,6-bisphosphatase deficiencies / frucyose-1,6-bisphosphatase deficiencies / fruchose-1,6-bisphosphatase deficiencies / frucgose-1,6-bisphosphatase deficiencies / frucfose-1,6-bisphosphatase deficiencies / frucrose-1,6-bisphosphatase deficiencies / fruc4ose-1,6-bisphosphatase deficiencies / fruct9se-1,6-bisphosphatase deficiencies / fruct0se-1,6-bisphosphatase deficiencies / fructpse-1,6-bisphosphatase deficiencies / fructlse-1,6-bisphosphatase deficiencies / fructkse-1,6-bisphosphatase deficiencies / fructise-1,6-bisphosphatase deficiencies / fruct8se-1,6-bisphosphatase deficiencies / fructowe-1,6-bisphosphatase deficiencies / fructoee-1,6-bisphosphatase deficiencies / fructode-1,6-bisphosphatase deficiencies / fructoxe-1,6-bisphosphatase deficiencies / fructoze-1,6-bisphosphatase deficiencies / fructoae-1,6-bisphosphatase deficiencies / fructoqe-1,6-bisphosphatase deficiencies / fructos3-1,6-bisphosphatase deficiencies / fructos4-1,6-bisphosphatase deficiencies / fructosr-1,6-bisphosphatase deficiencies / fructosf-1,6-bisphosphatase deficiencies / fructosd-1,6-bisphosphatase deficiencies / fructoss-1,6-bisphosphatase deficiencies / fructosw-1,6-bisphosphatase deficiencies / fructose=1,6-bisphosphatase deficiencies / fructose[1,6-bisphosphatase deficiencies / fructosep1,6-bisphosphatase deficiencies / fructose01,6-bisphosphatase deficiencies / fructose-2,6-bisphosphatase deficiencies / fructose-w,6-bisphosphatase deficiencies / fructose-q,6-bisphosphatase deficiencies / fructose-`,6-bisphosphatase deficiencies / fructose-1,7-bisphosphatase deficiencies / fructose-1,u-bisphosphatase deficiencies / fructose-1,y-bisphosphatase deficiencies / fructose-1,t-bisphosphatase deficiencies / fructose-1,5-bisphosphatase deficiencies / fructose-1,6=bisphosphatase deficiencies / fructose-1,6[bisphosphatase deficiencies / fructose-1,6pbisphosphatase deficiencies / fructose-1,60bisphosphatase deficiencies / fructose-1,6-visphosphatase deficiencies / fructose-1,6-fisphosphatase deficiencies / fructose-1,6-gisphosphatase deficiencies / fructose-1,6-hisphosphatase deficiencies / fructose-1,6-nisphosphatase deficiencies / fructose-1,6- isphosphatase deficiencies / fructose-1,6-bsphosphatase deficiencies / fructose-1,6-biwphosphatase deficiencies / fructose-1,6-biephosphatase deficiencies / fructose-1,6-bidphosphatase deficiencies / fructose-1,6-bixphosphatase deficiencies / fructose-1,6-bizphosphatase deficiencies / fructose-1,6-biaphosphatase deficiencies / fructose-1,6-biqphosphatase deficiencies / fructose-1,6-bis0hosphatase deficiencies / fructose-1,6-bis-hosphatase deficiencies / fructose-1,6-bis[hosphatase deficiencies / fructose-1,6-bis;hosphatase deficiencies / fructose-1,6-bislhosphatase deficiencies / fructose-1,6-bisohosphatase deficiencies / fructose-1,6-bis9hosphatase deficiencies / fructose-1,6-bispyosphatase deficiencies / fructose-1,6-bispuosphatase deficiencies / fructose-1,6-bispjosphatase deficiencies / fructose-1,6-bispnosphatase deficiencies / fructose-1,6-bispbosphatase deficiencies / fructose-1,6-bispgosphatase deficiencies / fructose-1,6-bisptosphatase deficiencies / fructose-1,6-bisph9sphatase deficiencies / fructose-1,6-bisph0sphatase deficiencies / fructose-1,6-bisphpsphatase deficiencies / fructose-1,6-bisphlsphatase deficiencies / fructose-1,6-bisphksphatase deficiencies / fructose-1,6-bisphisphatase deficiencies / fructose-1,6-bisph8sphatase deficiencies / fructose-1,6-bisphowphatase deficiencies / fructose-1,6-bisphoephatase deficiencies / fructose-1,6-bisphodphatase deficiencies / fructose-1,6-bisphoxphatase deficiencies / fructose-1,6-bisphozphatase deficiencies / fructose-1,6-bisphoaphatase deficiencies / fructose-1,6-bisphoqphatase deficiencies / fructose-1,6-bisphos0hatase deficiencies / fructose-1,6-bisphos-hatase deficiencies / fructose-1,6-bisphos[hatase deficiencies / fructose-1,6-bisphos;hatase deficiencies / fructose-1,6-bisphoslhatase deficiencies / fructose-1,6-bisphosohatase deficiencies / fructose-1,6-bisphos9hatase deficiencies / fructose-1,6-bisphospyatase deficiencies / fructose-1,6-bisphospuatase deficiencies / fructose-1,6-bisphospjatase deficiencies / fructose-1,6-bisphospnatase deficiencies / fructose-1,6-bisphospbatase deficiencies / fructose-1,6-bisphospgatase deficiencies / fructose-1,6-bisphosptatase deficiencies / fructose-1,6-bisphosphqtase deficiencies / fructose-1,6-bisphosphwtase deficiencies / fructose-1,6-bisphosphstase deficiencies / fructose-1,6-bisphosphxtase deficiencies / fructose-1,6-bisphosphztase deficiencies / fructose-1,6-bisphospha5ase deficiencies / fructose-1,6-bisphospha6ase deficiencies / fructose-1,6-bisphosphayase deficiencies / fructose-1,6-bisphosphahase deficiencies / fructose-1,6-bisphosphagase deficiencies / fructose-1,6-bisphosphafase deficiencies / fructose-1,6-bisphospharase deficiencies / fructose-1,6-bisphospha4ase deficiencies / fructose-1,6-bisphosphatqse deficiencies / fructose-1,6-bisphosphatwse deficiencies / fructose-1,6-bisphosphatsse deficiencies / fructose-1,6-bisphosphatxse deficiencies / fructose-1,6-bisphosphatzse deficiencies / fructose-1,6-bisphosphatawe deficiencies / fructose-1,6-bisphosphataee deficiencies / fructose-1,6-bisphosphatade deficiencies / fructose-1,6-bisphosphataxe deficiencies / fructose-1,6-bisphosphataze deficiencies / fructose-1,6-bisphosphataae deficiencies / fructose-1,6-bisphosphataqe deficiencies / fructose-1,6-bisphosphatas3 deficiencies / fructose-1,6-bisphosphatas4 deficiencies / fructose-1,6-bisphosphatasr deficiencies / fructose-1,6-bisphosphatasf deficiencies / fructose-1,6-bisphosphatasd deficiencies / fructose-1,6-bisphosphatass deficiencies / fructose-1,6-bisphosphatasw deficiencies / fructose-1,6-bisphosphatase eeficiencies / fructose-1,6-bisphosphatase reficiencies / fructose-1,6-bisphosphatase feficiencies / fructose-1,6-bisphosphatase veficiencies / fructose-1,6-bisphosphatase ceficiencies / fructose-1,6-bisphosphatase xeficiencies / fructose-1,6-bisphosphatase seficiencies / fructose-1,6-bisphosphatase weficiencies / fructose-1,6-bisphosphatase d3ficiencies / fructose-1,6-bisphosphatase d4ficiencies / fructose-1,6-bisphosphatase drficiencies / fructose-1,6-bisphosphatase dfficiencies / fructose-1,6-bisphosphatase ddficiencies / fructose-1,6-bisphosphatase dsficiencies / fructose-1,6-bisphosphatase dwficiencies / fructose-1,6-bisphosphatase dericiencies / fructose-1,6-bisphosphatase deticiencies / fructose-1,6-bisphosphatase degiciencies / fructose-1,6-bisphosphatase debiciencies / fructose-1,6-bisphosphatase deviciencies / fructose-1,6-bisphosphatase deciciencies / fructose-1,6-bisphosphatase dediciencies / fructose-1,6-bisphosphatase deeiciencies / fructose-1,6-bisphosphatase defciencies / fructose-1,6-bisphosphatase defixiencies / fructose-1,6-bisphosphatase defisiencies / fructose-1,6-bisphosphatase defidiencies / fructose-1,6-bisphosphatase defifiencies / fructose-1,6-bisphosphatase defiviencies / fructose-1,6-bisphosphatase defi iencies / fructose-1,6-bisphosphatase deficencies / fructose-1,6-bisphosphatase defici3ncies / fructose-1,6-bisphosphatase defici4ncies / fructose-1,6-bisphosphatase deficirncies / fructose-1,6-bisphosphatase deficifncies / fructose-1,6-bisphosphatase deficidncies / fructose-1,6-bisphosphatase deficisncies / fructose-1,6-bisphosphatase deficiwncies / fructose-1,6-bisphosphatase deficiebcies / fructose-1,6-bisphosphatase deficiehcies / fructose-1,6-bisphosphatase deficiejcies / fructose-1,6-bisphosphatase deficiemcies / fructose-1,6-bisphosphatase deficie cies / fructose-1,6-bisphosphatase deficienxies / fructose-1,6-bisphosphatase deficiensies / fructose-1,6-bisphosphatase deficiendies / fructose-1,6-bisphosphatase deficienfies / fructose-1,6-bisphosphatase deficienvies / fructose-1,6-bisphosphatase deficien ies / fructose-1,6-bisphosphatase deficiences / fructose-1,6-bisphosphatase deficienci3s / fructose-1,6-bisphosphatase deficienci4s / fructose-1,6-bisphosphatase deficiencirs / fructose-1,6-bisphosphatase deficiencifs / fructose-1,6-bisphosphatase deficiencids / fructose-1,6-bisphosphatase deficienciss / fructose-1,6-bisphosphatase deficienciws / fructose-1,6-bisphosphatase deficienciew / fructose-1,6-bisphosphatase deficienciee / fructose-1,6-bisphosphatase deficiencied / fructose-1,6-bisphosphatase deficienciex / fructose-1,6-bisphosphatase deficienciez / fructose-1,6-bisphosphatase deficienciea / fructose-1,6-bisphosphatase deficiencieq /