Glycogen Storage Diseases- Multiple Choice Questions

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1. A 3-month-old male infant presents with poor feeding, hypotonia, and cardiomegaly on physical exam. Blood tests reveal elevated lactate, uric acid, and creatine kinase levels. Echocardiogram demonstrates hypertrophic cardiomyopathy. A muscle biopsy shows excessive glycogen storage in lysosomes. Which enzyme deficiency is most likely responsible for this infant’s condition? A. Glucose-6-phosphatase B. Debranching enzyme […]

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Glycogen storage diseases with mnemonics to aid memorization

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A table summarizing the main glycogen storage diseases (GSDs) with enzyme defects, key clinical features, and mnemonics to aid memorization: Glycogen Storage Disease Enzyme Defect Key Clinical Features Mnemonic Type I: von Gierke Disease (GSD I) Glucose-6-phosphatase Severe fasting hypoglycemia, hepatomegaly, lactic acidosis, hyperlipidemia, hyperuricemia “von Gierke: ‘G6P’s Gone, Liver Left Low” Type II: Pompe

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Glycogen Metabolism- Multiple-Choice Questions with Complete explanations-Part-1

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1. Imagine you’re preparing for a long-distance marathon and need sustained energy throughout the race. Which of the following organs stores the maximum amount of glycogen that your body can use during prolonged exercise? A. Adipose tissue B. Cardiac muscle C. Kidney D. Liver E. Skeletal muscle   The correct answer is E. Skeletal muscle:

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Multiple-Choice Questions- HMP/Pentose phosphate pathway

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1. A researcher is investigating the effect of an enzyme whose reaction product is crucial for de novo fatty acid synthesis. Which of the following enzymes is most likely the focus of this study? A. Acyl CoA Dehydrogenase B. Glucose-6-Phosphatase C. Glucose-6-Phosphate Dehydrogenase (G-6PD) D. Lactate Dehydrogenase E. Phosphofructokinase-1 (PFK-1)   Correct Answer – C.

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Gluconeogenesis- Case-based multiple-choice questions- Set 2

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11. During fasting and feeding states, several enzymes are regulated to maintain glucose and energy homeostasis. Specific biochemical processes occur in an individual at rest who has fasted for 12 hours to adapt to the energy demand. Which of the following occurs in such a state? A. Phosphodiesterase is activated by insulin B. Pyruvate carboxylase

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Gluconeogenesis: Pathways, Substrates, and Key Enzymes Explained with mnemonics

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Gluconeogenesis is a vital metabolic process that allows the body to synthesize glucose from non-carbohydrate sources, ensuring a continuous supply of glucose, especially during fasting or intense exercise. Below, we explore how key substrates enter gluconeogenesis and the role of specific enzymes with easy-to-remember mnemonics to simplify learning.   Figure 1: Gluconeogenesis and Its Connection

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Gluconeogenesis- Case-based Multiple-Choice Questions- Set 1

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1. A 4-month-old boy is being evaluated for seizures, psychomotor retardation, and hypotonia. Work-up reveals elevated serum levels of lactate, alanine, pyruvate, and ketoacids. Based on the clinical presentation, pyruvate carboxylase activity is measured using fibroblasts from a skin biopsy and is found to be markedly decreased. This enzyme is normally used to directly synthesize

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