What is the effect of a defective a(1→ 4) phosphatase in Pompe's disease (GSD II)?O Accumulation of abnormal, unbranched glycogen, leading to heart or liver failure and short fife expectancyO Glycogen accumulation in heart, leading to heart failure, muscle weakness, short life expectancyO Absence of UDP-glucose conversion to glycogen, leading to lack of muscle glycogen, exercise intoleranceGlycogen accumulation due to lack of glucose-6-phosphate conversion to glucose, leading to liver enlargement,hypoglycemia, short life expectancyO Lack of complete degradation of glycogen to glucose. leading to mild hypoglycemia, enlarged liver and muscle weaknessSubmitRequest Answer

Glycogen Storage Disease type II , also called Pompe's disease occurs to due to the deficiency of…

Answered: What is the effect of a defective a(1→…

Biochemistry

6th Edition

ISBN:9781305577206

Author:Reginald H. Garrett, Charles M. Grisham

Publisher:Reginald H. Garrett, Charles M. Grisham

Chapter22: Gluconeogenesis, Glycogen Metabolism, And The Pentose Phosphate Pathway

Section: Chapter Questions

Problem 8P

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Understanding the Mechanism of Hemolytic Anemia Genetic defects in glycolytic enzymes can have serious consequences for humans For example, defects in the gene for pyruvate kinase can result in a condition known as hemolytic anemia. Consult a reference to learn about hemolytic anemia, and discuss why such genetic defects lead to this condition.
Radiolabeling with 14C-Glutamate Describe the labeling pattern that would result from the introduction into the TCA cycle of glutamate labeled at Cy with 14C.
Discuss the roles of phospholipase A2, cyclooxygenase, and lipooxygenase.
Fructose-2,6-bisphosphate is an activator of and an inhibitor of O phosphofructokinase; hexokinase O fructose bisphosphatase; phosphofructokinase O phosphofructokinase; fructose bisphosphatase O glucose-6-phosphatase; pyruvate kinase O pyruvate kinase; phosphoenolpyruvate carboxykinase
What is the advantage for some tumors to stop expressing the genes for glucose-6-phosphatase, phosphoenolpyruvate carboxykinase, and fructose bisphosphatase?
Von Gierke’s disease is also known as glycogen storage disease type I. Patients with von Gierke’s disease lackglucose 6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis (elevated lactate levels in the blood), especially during strenuous exercise. Explain why these symptoms occur. What chemical reaction does this enzyme catalyze? Which pathways involve this enzyme? Lacking thisthe enzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving Cori’s cycle. can you please do not write by your hand? I mean computer if you can. thank you
Which of the following is likely to be correct if all mutases are completely inhibited?a. Removal of glucosyl residues from nonreducing ends of glycogen in the liver is compromised;therefore, release of free glucose into blood from the liver would decreaseb. Glycerol can be efficiently converted into glucose but cannot be efficiently converted into glucosylresidues in glycogen in the liverc. Bothaandbd. Neither a nor b
Generally speaking, if glycogen phosphorylase is activated by being phosphorylated, then glycogen synthase is also activated by being phosphorylated. Select one: a. True b. False
Patients with von Gierke’s disease (a glycogen storage disease) lack glucose-6- phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis. Can you explain why these symptoms occur?
Indicate what will happen ( increase, decrease or no effect) tothe activity of the enzyme or rate of the metabolic pathway in the given conditions a. release of glucagon in the blood to the activity of carnitine acyl transferase 1 b. high malonyl CoA to the activity of carnitine acyl transferase I C. Epinephrine to the activity og glycogen synthase d. high citrate to the activity of acetyl CoA carboxylase e. high acetyl CoA to ketogenesis
In muscle tissue, the rate of conversion of glycogen to glucose 6-phosphate is determined by the ratio of phosphorylase a (active) to phosphorylase b (less active). Determine what happens to the rate of glycogen breakdown if a muscle preparation containing glycogen phosphorylase is treated with: (a) phosphorylase kinase and ATP; (b) PP1; (c) epinephrine
Glycogen synthase may be regulated by covalent modification and/or allosteric control. Label the diagram with the appropriate terms to describe glycogen synthase regulation. Use the following options: Glucose-6-Phospate; Insulin; Fructose-2,6-Bisphosphate; Phosphorylation, Dephosphorylation

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