Carbohydrate Metabolism — MCQs

Carbohydrate Metabolism Indian Medical PG Practice Questions and MCQs

Question 771: Which glycogen storage disease also presents as a lysosomal storage disease?

A. Von Gierke's disease
B. McArdle's disease
C. Andersen's disease
D. Pompe's disease (Correct Answer)

Explanation: ***Pompe's disease*** - Also known as **glycogen storage disease type II**, it is caused by a deficiency of **acid alpha-glucosidase (GAA)**, a *lysosomal enzyme*. - This deficiency leads to the accumulation of **glycogen in lysosomes**, particularly affecting muscle tissue, thereby earning its classification as both a glycogen storage disease and a lysosomal storage disease. *Von Gierke's disease* - This is **glycogen storage disease type I** and is due to a deficiency in **glucose-6-phosphatase**. - It primarily affects the **liver and kidneys**, causing severe **hypoglycemia** and **lactic acidosis**, but it is not classified as a lysosomal storage disease. *McArdle's disease* - This is **glycogen storage disease type V**, caused by a deficiency in **muscle glycogen phosphorylase (myophosphorylase)**. - It manifests as **exercise intolerance** and muscle pain, but it does not involve lysosomal enzyme defects or glycogen accumulation in lysosomes. *Andersen's disease* - This is **glycogen storage disease type IV**, caused by a deficiency in the **glycogen branching enzyme**. - It leads to the formation of **abnormal glycogen structures**, primarily affecting the liver and causing early liver failure, but it is not a lysosomal storage disorder.

Question 772: Increased uric acid levels are seen in which glycogen storage disease ?

A. Type I (Von Gierke's disease) (Correct Answer)
B. Type II (Pompe disease)
C. Type IV (Andersen disease)
D. Type III (Cori disease)

Explanation: ***Type I (Von Gierke's disease)*** - In **Von Gierke's disease**, the deficiency of **glucose-6-phosphatase** leads to accumulation of glucose-6-phosphate in hepatocytes. - **Hyperuricemia** occurs due to: 1. **Increased purine degradation** - Metabolic stress leads to accelerated ATP breakdown and increased uric acid production 2. **Decreased renal excretion** - Lactic acidosis (from G6P → pyruvate → lactate) competitively inhibits uric acid secretion in renal tubules 3. **Enhanced purine synthesis** - Increased availability of ribose-5-phosphate from pentose phosphate pathway - Classic triad: **Hepatomegaly, hypoglycemia, and lactic acidosis with hyperuricemia** *Type II (Pompe disease)* - Caused by a deficiency of **acid alpha-glucosidase** (acid maltase), leading to glycogen accumulation in **lysosomes**. - Primarily affects the **heart**, **muscles**, and **liver**, but does not cause hyperuricemia. *Type IV (Andersen disease)* - Results from a deficiency of **glycogen branching enzyme**, leading to the formation of abnormal glycogen with long, unbranched chains. - Primarily affects the **liver** and **spleen**, causing cirrhosis and hepatic failure, but not hyperuricemia. *Type III (Cori disease)* - Caused by a deficiency of **glycogen debranching enzyme** (amylo-1,6-glucosidase), leading to abnormal accumulation of glycogen with short outer branches. - Presents with hepatomegaly, hypoglycemia, and muscle weakness, but **hyperuricemia is not a characteristic feature**.

Question 773: GLUT2 plays a functionally important role mainly in?

A. Liver
B. Pancreatic beta cells (Correct Answer)
C. Skeletal muscle tissue
D. Kidney

Explanation: ***Pancreatic beta cells*** - **GLUT2** acts as a **glucose sensor** in pancreatic beta cells, which is its **most functionally critical role** in the body. - Its high Km (~15-20 mM, low affinity) ensures that glucose uptake is **proportional to blood glucose concentration**, enabling the beta cells to accurately sense glucose levels and secrete insulin accordingly. - This glucose-sensing mechanism is **essential for maintaining glycemic homeostasis** and makes GLUT2's role in beta cells uniquely important compared to its presence in other tissues. - Without functional GLUT2 in beta cells, the body cannot properly regulate insulin secretion in response to changing glucose levels. *Liver* - While **GLUT2** is abundantly expressed in hepatocytes and allows for bidirectional glucose transport (both uptake and release), its role here is **facilitative rather than regulatory**. - The liver has multiple other glucose-regulating mechanisms (glucokinase, glucose-6-phosphatase, glycogen metabolism). - GLUT2's function in the liver is important but not as uniquely critical as its glucose-sensing role in beta cells. *Skeletal muscle tissue* - **Skeletal muscle** primarily utilizes **GLUT4** (not GLUT2) for insulin-dependent glucose uptake. - **GLUT2** is not significantly expressed in skeletal muscle tissue. - This makes GLUT2 functionally unimportant in skeletal muscle. *Kidney* - The **kidney** expresses **GLUT2** in proximal tubule cells where it participates in glucose reabsorption from the glomerular filtrate. - However, this role is **secondary to SGLT2** (sodium-glucose cotransporter 2), which performs the primary active reabsorption. - GLUT2's function here is important but not the **"mainly"** critical role compared to its glucose-sensing function in beta cells.

Question 774: Enzymes of glycolysis are found in:

A. Cytosol (Correct Answer)
B. Cell membrane
C. Mitochondria
D. Ribosomes

Explanation: ***Cytosol*** - Glycolysis is a metabolic pathway that occurs in the **cytosol** of cells. - All the enzymes required for the conversion of glucose to pyruvate are freely dissolved in the **cytoplasm**. *Cell membrane* - The cell membrane is primarily involved in **regulating the passage of substances** into and out of the cell, as well as cell signaling. - Glycolytic enzymes are not associated with the cell membrane. *Mitochondria* - Mitochondria are the primary site of **oxidative phosphorylation** and the **citric acid cycle**, not glycolysis. - While pyruvate (the end product of glycolysis) moves into the mitochondria for further metabolism, the initial glycolytic steps do not occur there. *Ribosomes* - Ribosomes are responsible for **protein synthesis** (translation). - They do not contain enzymes for metabolic pathways like glycolysis.

Question 775: Which of the following is an amino sugar formed from fructose-6-phosphate?

A. N-acetylglucosamine-6-phosphate
B. Glucosamine-6-phosphate (Correct Answer)
C. Galactosamine-6-phosphate
D. UDP-N-acetylglucosamine

Explanation: ***Glucosamine-6-phosphate*** - This amino sugar is directly synthesized from **fructose-6-phosphate** via a transamidation reaction, where an amino group replaces a hydroxyl group. - It is a key intermediate in the biosynthesis of other **amino sugars** and **glycosaminoglycans**. *N-acetylglucosamine-6-phosphate* - This is formed from **glucosamine-6-phosphate** by the addition of an **acetyl group**, making it a subsequent product, not the initial amino sugar from fructose-6-phosphate. - The N-acetylation step is crucial for its role in cellular signaling and structural components. *Galactosamine-6-phosphate* - While an amino sugar, **galactosamine-6-phosphate** is derived from UDP-N-acetylglucosamine, not directly from fructose-6-phosphate. - Its formation involves an **epimerization** step of an existing N-acetylglucosamine structure. *UDP-N-acetylglucosamine* - This is an **activated form** of N-acetylglucosamine, formed by the addition of UTP to N-acetylglucosamine-1-phosphate. - It serves as a precursor for the synthesis of complex **carbohydrates** and glycoproteins, far downstream from fructose-6-phosphate.

Question 776: At which step in glycolysis is NADH produced during the oxidation of glyceraldehyde-3-phosphate?

A. Pyruvate kinase
B. Enolase
C. PFK-1
D. Glyceraldehyde-3-phosphate dehydrogenase (Correct Answer)

Explanation: ***Glyceraldehyde-3-phosphate dehydrogenase*** - This enzyme catalyzes the oxidation and **phosphorylation** of glyceraldehyde-3-phosphate, producing **1,3-bisphosphoglycerate**. - During this reaction, **NAD+ is reduced to NADH**, which is a crucial step for energy production. *Pyruvate kinase* - This enzyme catalyzes the final step of glycolysis, transferring a phosphate group from **phosphoenolpyruvate** to ADP, forming ATP and pyruvate. - This step involves **substrate-level phosphorylation** for ATP production, not NADH. *Enolase* - This enzyme catalyzes the dehydration of **2-phosphoglycerate** to form **phosphoenolpyruvate (PEP)**. - This reaction involves the removal of a water molecule and does not produce NADH. *PFK-1* - **Phosphofructokinase-1 (PFK-1)** catalyzes the phosphorylation of fructose-6-phosphate to **fructose-1,6-bisphosphate**. - This is an ATP-consuming and a crucial regulatory step in glycolysis, but it does not involve NADH production.

Question 777: Which carbohydrate is primarily metabolized by Aldolase-B?

A. Galactose
B. Fructose (Correct Answer)
C. Sucrose
D. None of the options

Explanation: ***Fructose*** - **Aldolase B** is a key enzyme in the liver responsible for the metabolism of **fructose**, specifically cleaving **fructose-1-phosphate** into **dihydroxyacetone phosphate** and **glyceraldehyde**. - A deficiency in **Aldolase B** leads to **hereditary fructose intolerance**, causing an accumulation of **fructose-1-phosphate** after fructose ingestion. *Galactose* - **Galactose** is primarily metabolized by enzymes in the **Leloir pathway**, including **galactokinase** and **galactose-1-phosphate uridylyltransferase**. - **Aldolase B** plays no significant role in the metabolism of galactose. *Sucrose* - **Sucrose** is a disaccharide composed of **glucose** and **fructose**. - It is first broken down by **sucrase** in the small intestine into its constituent monosaccharides before they are metabolized further. *None of the options* - This option is incorrect because **fructose** is indeed a carbohydrate primarily metabolized by Aldolase-B. - The enzyme's specific role in fructose metabolism is well-established.

Question 778: Which of the following GAG is not sulfated?

A. Keratan sulfate
B. Dermatan sulfate
C. Chondroitin sulfate
D. Hyaluronic acid (Correct Answer)

Explanation: ***Hyaluronic acid*** - **Hyaluronic acid** is unique among glycosaminoglycans (GAGs) because it is the only one that is **not sulfated**. - It also distinguishes itself by being the only GAG that does **not form proteoglycans** and is not synthesized in the Golgi apparatus. *Chondroitin sulfate* - **Chondroitin sulfate** is a sulfated glycosaminoglycan that is a major component of the **extracellular matrix**, particularly in cartilage. - Its sulfate groups contribute to its **negative charge**, allowing it to attract water and provide resistance to compression. *Dermatan sulfate* - **Dermatan sulfate** is another sulfated GAG, found predominantly in the skin, blood vessels, and heart valves. - It contains **sulfate groups**, which are crucial for its interactions with various proteins and its role in tissue structure. *Keratan sulfate* - **Keratan sulfate** is a sulfated GAG found in the cornea, cartilage, and bone. - It is distinct from other GAGs due to its **lack of uronic acid** and the presence of sulfate groups.

Question 779: What cofactor is required for the proper functioning of glucose-6-phosphate dehydrogenase?

A. NAD
B. NADP (Correct Answer)
C. FAD
D. FMN

Explanation: ***NADP*** - **NADP+** (nicotinamide adenine dinucleotide phosphate) acts as the **electron acceptor** in the **glucose-6-phosphate dehydrogenase (G6PD)** reaction, becoming **NADPH**. - **NADPH** is crucial for maintaining the **redox balance** in cells, particularly in red blood cells, by reducing **oxidative stress**. *NAD* - **NAD+** (nicotinamide adenine dinucleotide) is a primary cofactor for many **dehydrogenase reactions** in catabolic pathways like **glycolysis** and the **Krebs cycle**. - It primarily functions as an electron acceptor in pathways that generate **ATP**, distinct from the role of **NADPH** in reductive biosynthesis and antioxidant defense. *FAD* - **FAD** (flavin adenine dinucleotide) is a coenzyme derived from **riboflavin (vitamin B2)** that is involved in various redox reactions, often in the form of **flavoproteins**. - Enzymes like **succinate dehydrogenase** in the electron transport chain utilize **FAD** as an electron acceptor, which is not the case for G6PD. *FMN* - **FMN** (flavin mononucleotide) is another coenzyme derived from **riboflavin**, structurally similar to FAD but lacking the additional adenosine monophosphate. - It participates in electron transfer reactions, particularly within **complex I** of the **electron transport chain**, but is not a cofactor for G6PD.

Question 780: Which enzyme is active when the insulin:glucagon ratio is low?

A. Fructokinase
B. Lactate dehydrogenase
C. Acetyl-CoA carboxylase
D. Glucose-6-phosphatase (Correct Answer)

Explanation: ***Glucose-6-phosphatase*** - A low **insulin:glucagon ratio** occurs during **fasting, starvation, or stress** (catabolic state). - In this metabolic state, **glucose-6-phosphatase** is **highly active** in the **liver** (and kidneys), catalyzing the final step of both **gluconeogenesis** and **glycogenolysis**. - It converts **glucose-6-phosphate** to **free glucose**, which is released into the bloodstream to maintain **blood glucose levels**. - This enzyme is stimulated by **glucagon** and inhibited by **insulin**. *Fructokinase* - This enzyme phosphorylates **fructose** to **fructose-1-phosphate** in the liver. - It is regulated by **fructose availability**, not by the **insulin:glucagon ratio**. - During a low insulin:glucagon state, **gluconeogenic** and **glycogenolytic** pathways are favored, not fructose metabolism. *Lactate dehydrogenase* - **LDH** interconverts **pyruvate** and **lactate** and functions in both **glycolysis** and **gluconeogenesis**. - While it plays a role in various metabolic states, it is **not specifically activated** by a low insulin:glucagon ratio. - It operates constitutively based on substrate availability rather than hormonal regulation. *Acetyl-CoA carboxylase* - This is the **rate-limiting enzyme** of **fatty acid synthesis** (lipogenesis). - It is **activated by insulin** (fed state) and **inhibited by glucagon** (fasted state). - When the insulin:glucagon ratio is **low**, this enzyme is **phosphorylated and INACTIVE**, shutting down fatty acid synthesis.

Practice by Chapter

Carbohydrate Chemistry and Classification

Practice Questions

Glycolysis: Reactions and Regulation

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Gluconeogenesis: Reactions and Regulation

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Glycogen Metabolism: Synthesis and Breakdown

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Glycogen Storage Diseases

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Pentose Phosphate Pathway

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Metabolism of Fructose and Galactose

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Disorders of Fructose and Galactose Metabolism

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Blood Glucose Regulation

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Diabetes Mellitus: Biochemical Aspects

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Glycosylation and Glycoproteins

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Lactose Intolerance and Galactosemia

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