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Fructose, metabolism deficiencies

Deficiency in fructose metabolism, as in essential fme-tosuria, may be perfectly benign, for one is not interrupting the main line of the glycolytic chain. However, there is one condition, hereditary fructose intolerance, which does cause significant problems. [Pg.50]

Fructose-1,6-bisphosphatase deficiency, first describ ed by Baker and Winegrad in 1970, has now been reported in approximately 30 cases. It is more common in women and is inherited as an autosomal recessive disorder. Initial manifestations are not strikingly dissimilar from those of glucose-6-phosphatase deficiency. Neonatal hypoglycemia is a common presenting feature, associated with profound metabolic acidosis, irritability or coma, apneic spells, dyspnea, tachycardia, hypotonia and moderate hepatomegaly. Lactate, alanine, uric acid and ketone bodies are elevated in the blood and urine [11]. The enzyme is deficient in liver, kidney, jejunum and leukocytes. Muscle fructose-1,6-bisphosphatase activity is normal. [Pg.704]

Baker L, Winegrad AI (1970) Fasting hypoglycaemia and metabolic acidosis associated with deficiency of hepatic fructose-1,6-diphosphatase activity. Lancet ii 13 16... [Pg.469]

Fig. 8.2 Glycolysis and related pathways. Glycolysis is a central metabolic machinery in which one mole of glucose is catabolized to two moles of pyruvate, NADH, and ATP. Under aerobic conditions, pyruvate is further oxidized by mitochondrial system. In erythrocytes DHAP is a dead-end product however, in brain it can be converted into direction of lipid synthesis. Glycolysis and the pentose phosphate pathway (pentosePP) are interconnected via fructose-6-P and glyceral-dehyde-3-P. A high level of NADPH favors lipid synthesis via pentose phosphate shunt (pentosePP). At TPI inhibition (TPI deficiency), glyceraldehyde-3-Pcan be produced via G6PDH as well, to contribute to the glycolytic flux. a-GDH catalyzes the... Fig. 8.2 Glycolysis and related pathways. Glycolysis is a central metabolic machinery in which one mole of glucose is catabolized to two moles of pyruvate, NADH, and ATP. Under aerobic conditions, pyruvate is further oxidized by mitochondrial system. In erythrocytes DHAP is a dead-end product however, in brain it can be converted into direction of lipid synthesis. Glycolysis and the pentose phosphate pathway (pentosePP) are interconnected via fructose-6-P and glyceral-dehyde-3-P. A high level of NADPH favors lipid synthesis via pentose phosphate shunt (pentosePP). At TPI inhibition (TPI deficiency), glyceraldehyde-3-Pcan be produced via G6PDH as well, to contribute to the glycolytic flux. a-GDH catalyzes the...
DHAP is a glycolysis intermediate, whereas glyceraldehyde must be reduced by a mitochondrial enzyme, glyceraldehyde dehydrogenase, to glycerol, which is then subject to action by glycerol kinase in the liver. The aldolase seems to be the principal pathway of metabolizing fructose and depends on the initial phosphorylation step catalyzed by fructokinase, which produces fructose-l-phosphate. Fructokinase is defective in an inherited disorder, essential fructosuria. Fructose-l-phosphate aldolase is deficient in the hereditary disorder fructose intolerance. [Pg.487]

Endogenous hepatosis comprises endogenous metabolic disorders of the liver cell as a rule, the terminology used in this context refers to the accumulated substances (e. g. glycogenosis), the harmful substrate (e.g. fructose-1 phosphate) or the enzyme defect (e.g. apantitrypsin deficiency). [Pg.405]

Fructokinase is deficient in essential fructosuria therefore, fructose can-s not be metabolized as rapidly as it normally would. Blood fructose levels... [Pg.174]

The toxic effects of FIP can also be exhibited in patients that do not have a deficiency in aldolase B if they are parenterally fed with solutions containing fructose. Parenteral feeding with solutions containing fructose can result in blood fructose concentrations that are several times higher than can be achieved with an oral load. Since the rate of entry into the hepatocyte is dependent on the fructose gradient across the cell, intravenous loading results in increased entry into the liver and increased formation of FIP. Since the rate of formation of FIP is much faster than its further metabolism, this can lead to hyperuricemia and hyperuricosuria by the mechanisms described above. [Pg.221]

B. Because the patient s liver enzymes are normal and her symptoms seem to correlate with her intake of fruit juices, most likely her problem stems from an inability to absorb fructose. Since removal of cow s milk from her diet did not eliminate the problem, a lactase deficiency can be ruled out. GLUT 5 is the primary transporter of fructose in the intestine and a deficiency in this transporter would lead to an inability to absorb fructose in the gut, making it a substrate for bacterial metabolism that produces various gases, including hydrogen, as well as organic acids. [Pg.223]


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See also in sourсe #XX -- [ Pg.50 ]




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