An adolescent male patient presented with pain in the calf muscles on exercise. On biopsy,an excessive amount of glycogen was found to be present in the muscle. What is the mostlikely enzyme deficiency?

In the given question, patient presents with pain in the calf muscle on exercise and excessive…

Answered: An adolescent male patient presented…

Biochemistry

9th Edition

ISBN:9781319114671

Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.

Chapter1: Biochemistry: An Evolving Science

Section: Chapter Questions

Problem 1P

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After the training of marathon, lactic acid is accumulated in the athlete's thigh muscle. Compare the physiological functions and Vmax of glycogen phosphorylase in actively working skeletal muscle and that in liver After vigorous exercise, the athlete faints due to low blood glucose level. A liver biopsy test shows excess amount of glycogen is present in the liver. Suggest with explanation, which type of enzyme is defective in this case.
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
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 thisenzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving the Cori’s cycle.
Lactic acid builds up in the athlete's thigh muscle after marathon training. Compare and contrast the physiological functions and Vmax of glycogen phosphorylase in active skeletal muscle and liver. The athlete faints after a rigorous workout owing to a low blood glucose level. A liver biopsy reveals that there is an excess of glycogen in the liver. Suggestions on whatever sort of enzyme is faulty in this circumstance should be accompanied by an explanation.
Explain why people with a hereditary deficiency of carnitine acyltransferase II have muscle weakness. Why are the symptoms more severe during fasting?
Sheryl had an elevated level of the CK-MB isoenzyme of creatine phosphokinase.What are the different isoenzymes of CK, and what is their medical significance?Explain how enzymes are named, and how isoenzymatic forms of a particular enzyme are the same, yet differ from each other.
A sample of glycogen from a patient with liver disease is incubated with orthophosphate, phosphorylase, the transferase, and the debranching enzyme ( α -1,6- glucosidase). The ratio of glucose 1- phosphate to glucose formed in this mixture is 100. What is the most likely enzymatic deficiency in this patient?
What is alpha keto glutarate dehydrogenase complex?. explain very briefly.
What is the effect of the cholesterol diet on the amount of HMGCoA reductase protein?
A generalized enzyme active site is shaped like a hemisphere with a radius of 45Å. The active site holds the following amino acids in a homeostatic solution (pH = 7.38): -HAVARILKHAVARILKHAVARILK- Assuming the charge is distributed uniformly along the hemisphere, determine the force at which this active site acts upon a single ATP molecule at the center of the hemisphere.
In the majority of cases, amino acid catabolism can be broken down into a two-stepprocess; ‘transamination’ followed by ‘oxidative deamination’. Serine doesn’t followthis two-step process; fully explain the reaction and mechanism involved in thedegradation process used to form its α-keto acid.
The clinical signs of two types of galactosemia—galactokinase deficiency or UDPglucose: galactose 1-phosphate uridylyltransferase deficiency—are markedly different. Although both forms cause gastrointestinal pain after drinking milk, transferase deficiency also causes liver, kidney, spleen, and brain malfunction, as well as mortality. With each kind of enzyme shortage, what products accumulate in the blood and tissues? Estimate the relative toxicity of these goods based on the information provided above.

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An adolescent male patient presented with pain in the calf muscles on exercise. On biopsy, an excessive amount of glycogen was found to be present in the muscle. What is the most likely enzyme deficiency?