Individuals with galactosemia A) None of the following B) lack the enzyme hexokinase which is required for phosphorylation of galactose. C) lack the enzyme galactokinase which is required for interconversion between glucose-1-phosphate and galactose-1-phosphate. D) cannot synthesize galactose from glucose. E) lack adequate uridine diphosphosphate.
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Galactosemia is a metabolic disorder in which the body cannot process the sugar galactose properly due to a deficiency of certain enzymes involved in its metabolism. Show more…
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Ability to correctly metabolize galactose for use in diseases caused by genetic defects that affect an individual's galactosemia is lactosemia. This results in high galactose levels in the blood and accumulation of galactose, but it has no effect on the metabolism of starch and glycogen or on glycolysis in the glycolytic pathway. Which of the following enzymes would you expect to be defective in a person with galactosemia? a) Uridyl transferase b) Hexokinase c) UDP-galactose 4-epimerase d) Fructose-1,6-bisphosphatase e) Pyruvate kinase
Madhur L.
Galactosemia can result from a deficiency of either galactokinase or UDP-glucose:galactose 1-phosphate uridylytransferase (transferase). Although a deficiency of either enzyme will cause gastric discomfort after milk ingestion, transferase deficiency also results in liver, kidney, spleen, and brain dysfunction, and in some cases, death. Select the statements that correctly link each type of enzyme deficiency with the metabolites that accumulate in the blood and tissues. A deficiency in either enzyme will result in an accumulation of lactose; however, a transferase deficiency will also cause an accumulation of the toxic product UDP-galactose. Transferase converts galactose 1-phosphate to UDP-galactose; therefore, a transferase deficiency will cause an accumulation of galactose 1-phosphate. A galactokinase deficiency will result in an accumulation of galactose 1-phosphate. Galactokinase catalyzes the formation of galactose 1-phosphate; therefore, a deficiency in galactokinase will result in an accumulation of galactose. A transferase deficiency will cause an accumulation of UDP-glucose.
Sri K.
16. Muscle glycogen cannot contribute directly to blood glucose levels because: A. Muscle glycogen cannot be converted to glucose-6-phosphate B. Muscle lacks glucose-6-phosphatase C. Muscle contains no glycogen phosphorylase D. Muscle lacks phosphoglucoisomerase 17. Which one of the following tissues requires insulin for glucose transport into the cell? A. Brain B. Liver C. Skeletal muscle D. Red blood cells 18. The mobilization of triacylglycerol-bound fatty acids from adipose tissue and their subsequent oxidation by muscle mitochondria require all of the following compounds except: A. Serum albumin B. Carnitine C. Acetyl CoA carboxylase D. Hormone-sensitive lipase 19. Which one of the following reactions is unique to gluconeogenesis? A. Lactate -> pyruvate B. Phosphoenolpyruvate -> pyruvate C. Oxaloacetate -> phosphoenolpyruvate D. 1,3-bisphosphoglycerate -> 3-phosphoglycerate 20. A newborn vomits after each feeding of milk-based formula and doesn't gain weight. Biochemical testing reveals a severe deficiency of galactose-1-phosphate uridyltransferase. If this condition goes untreated, which of the following is the likely outcome for this patient? A. Benign disease except for cataract formation B. Chronic emphysema appearing in early adulthood C. Chronic renal failure appearing in adolescence D. Death in infancy E. Gastrointestinal symptoms that remit with puberty
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