Carbohydrate Metabolism Practice Questions

Q: What is the cause of lactose intolerance?

Deficiency of Lactase. ***Deficiency of Lactase*** - Lactose intolerance results from the insufficient production of the enzyme **lactase**, which is responsible for breaking down **lactose** (a disaccharide found in milk and dairy products) into glucose and galactose. - When lactase is deficient, undigested lactose passes into the colon, where it is fermented by bacteria, leading to symptoms like **bloating**, **gas**, **diarrhea**, and **abdominal pain**. *Deficiency of Galactokinase* - A deficiency in **galactokinase** causes **Type II galactosemia**, a disorder involving the inability to metabolize galactose. - This condition primarily leads to **cataracts** and does not directly cause the digestive symptoms associated with lactose intolerance. *Deficiency of Uridyl transferase* - A deficiency in **uridyl transferase** causes **classic galactosemia (Type I)**, the most severe form of galactosemia. - This condition results in a buildup of toxic galactose metabolites, leading to **liver damage**, **renal failure**, and **developmental delay**, not lactose intolerance. *Deficiency of Enteropeptidase* - **Enteropeptidase** (also known as enterokinase) is an enzyme in the small intestine that activates trypsinogen to trypsin, which then activates other pancreatic proteases. - A deficiency leads to **protein malabsorption** and failure to thrive, not the fermentation of lactose by gut bacteria.

Q: Which of the following is an aldose sugar?

Glyceraldehyde. ***Glyceraldehyde*** - **Glyceraldehyde** is the simplest **aldose**, a monosaccharide with an **aldehyde group** (CHO) at one end of its carbon chain. - Its chemical structure is a three-carbon chain with the aldehyde group on the first carbon, making it an **aldo sugar**. *Ribulose* - **Ribulose** is a **ketose**, specifically a **ketopentose**, meaning it is a five-carbon sugar with a **ketone group** (C=O) in its structure. - The ketone group in ribulose is typically located on the second carbon, distinguishing it from aldoses. *Fructose* - **Fructose** is another example of a **ketose**, specifically a **ketohexose**, as it is a six-carbon sugar containing a **ketone group**. - Its ketone group is usually found on the second carbon atom, which differentiates ketoses from aldoses structurally. *None of the options* - This option is incorrect because **glyceraldehyde** is indeed an aldose sugar, fitting the definition of a monosaccharide with an aldehyde functional group. - As **glyceraldehyde** is correctly identified as an aldose, this choice would contradict the chemical classification of sugars.

Q: An adolescent male patient presents to you with exercise intolerance. He gives a history of developing cramps on exertion. Which of the following enzyme deficiencies could be the cause?

Myophosphorylase. ***Myophosphorylase*** - A deficiency in **myophosphorylase** (McArdle's disease, Glycogen Storage Disease Type V) impairs muscle glycogen breakdown, leading to **exercise intolerance** and **muscle cramps** due to insufficient ATP production during exertion. - Patients often experience a "second wind" phenomenon where symptoms improve after resting, as free fatty acids become an alternative fuel source. *Hexokinase* - A deficiency in **hexokinase** would affect the first step of glycolysis, impacting glucose phosphorylation in all tissues, not specifically causing exercise-induced muscle cramps. - This deficiency is rare and typically presents with **hemolytic anemia** due to impaired erythrocyte metabolism. *Glucose-6-phosphatase* - A deficiency in **glucose-6-phosphatase** (Von Gierke's disease, Glycogen Storage Disease Type Ia) primarily affects the liver and kidneys, leading to **fasting hypoglycemia**, lactic acidosis, and hepatomegaly, not exercise intolerance. - Muscle glycogen metabolism is unaffected in this condition. *Hepatic glycogen phosphorylase* - A deficiency in **hepatic glycogen phosphorylase** (Hers' disease, Glycogen Storage Disease Type VI) mainly causes **hepatomegaly** and **mild hypoglycemia** because the liver cannot effectively mobilize its glycogen stores. - **Muscle glycogen metabolism** remains normal, so exercise intolerance and cramps are not characteristic symptoms.

Q: When the insulin to glucagon ratio decreases, which enzyme is primarily active?

Glycogen phosphorylase. ***Glycogen phosphorylase*** - A decrease in the **insulin-to-glucagon ratio** indicates a **low blood glucose** state, signaling the need for glucose production. - **Glycogen phosphorylase** is the key enzyme in **glycogenolysis**, which breaks down stored glycogen into glucose-1-phosphate, thereby elevating blood glucose. - This is the **primary and fastest response** to decreased insulin/glucagon ratio. *Fructose-1,6-bisphosphatase* - This enzyme is crucial for **gluconeogenesis**, specifically catalyzing the dephosphorylation of **fructose-1,6-bisphosphate** to **fructose-6-phosphate**. - While active during low insulin/high glucagon states, it is involved in synthesizing glucose, not directly breaking down stored glycogen as quickly as glycogen phosphorylase. *Pyruvate carboxylase* - This enzyme is the first committed step in **gluconeogenesis**, converting **pyruvate to oxaloacetate** in the mitochondria. - Although active in response to a low insulin-to-glucagon ratio, its role is in synthesizing glucose from non-carbohydrate precursors, which is a slower process than immediate glycogen breakdown. *Glucose-6-phosphatase* - This enzyme is found primarily in the **liver and kidneys** and is responsible for dephosphorylating **glucose-6-phosphate** to free glucose, allowing it to exit the cell into the bloodstream. - While essential for the release of glucose from both gluconeogenesis and glycogenolysis, it acts at a later stage to make glucose available rather than initiating the breakdown of glycogen itself.

Q: Which one of the following shows allosteric inhibition of glycolysis?

Citrate. ***Citrate*** - **Citrate** is a classic **allosteric inhibitor** of **phosphofructokinase-1 (PFK-1)**, the key regulatory enzyme in glycolysis - It binds to an allosteric site (distinct from the active site), reducing PFK-1's affinity for **fructose-6-phosphate** - This is a **negative feedback mechanism** - when citrate accumulates (indicating sufficient ATP production via the citric acid cycle), glycolysis slows down *Malonic acid* - **Malonic acid** is a **competitive inhibitor** (NOT allosteric) of succinate dehydrogenase in the citric acid cycle - It structurally resembles succinate and competes for the active site directly *2,3-BPG* - **2,3-Bisphosphoglycerate (2,3-BPG)** is an **allosteric effector** of hemoglobin (decreases oxygen affinity), not an enzyme inhibitor in glycolysis - It binds to hemoglobin, not to glycolytic enzymes *Amino acid alanine* - **Alanine** is an allosteric inhibitor of **pyruvate kinase** (not a glycolytic regulator in this context) - While it does show allosteric inhibition, it acts on gluconeogenesis regulation in the liver, not as a direct glycolytic inhibitor

Q: Classical galactosemia (Type I) is due to deficiency of which enzyme?

Galactose-1-phosphate uridyltransferase. ***Galactose-1-phosphate uridyltransferase*** - **Galactosemia Type I** (**classical galactosemia**) is caused by a deficiency in **galactose-1-phosphate uridyltransferase (GALT)**. - This enzyme is crucial for converting **galactose-1-phosphate** to **glucose-1-phosphate** in the Leloir pathway of galactose metabolism. *Adenine phosphoribosyltransferase (APRT)* - Deficiency in **adenine phosphoribosyltransferase (APRT)** leads to **APRT deficiency**, characterized by **kidney stones** composed of 2,8-dihydroxyadenine. - This enzyme is involved in **purine salvage pathways**, not carbohydrate metabolism. *Fructose-1,6-bisphosphatase* - A deficiency in **fructose-1,6-bisphosphatase** causes **fructose-1,6-bisphosphatase deficiency**, a disorder of **gluconeogenesis**. - It results in **hypoglycemia** and **lactic acidosis**, especially during fasting. *Hexokinase* - **Hexokinase** phosphorylates glucose to **glucose-6-phosphate**, the first step in glycolysis. - Deficiency is rare but can lead to **nonspherocytic hemolytic anemia**.

Q: Enzyme deficient in Hers disease -

Liver phosphorylase. ***Liver phosphorylase*** - Hers disease, also known as Glycogen Storage Disease Type VI, is specifically caused by a deficiency of **liver phosphorylase**. - This enzyme is crucial for the breakdown of **glycogen in the liver**, leading to an inability to release glucose into the bloodstream during fasting. *Muscle phosphorylase* - Deficiency of **muscle phosphorylase** (myophosphorylase) causes **McArdle disease** (Glycogen Storage Disease Type V), which primarily affects muscle energy. - Patients typically present with exercise intolerance, muscle pain, and cramps, not the hepatic symptoms seen in Hers disease. *Acid maltase* - Deficiency of **acid maltase** (also known as alpha-glucosidase) is responsible for **Pompe disease** (Glycogen Storage Disease Type II), a lysosomal storage disorder. - This enzyme deficiency leads to glycogen accumulation in lysosomes in various tissues, including muscle, liver, and heart, causing muscle weakness and cardiomyopathy. *Debranching enzyme* - A deficiency in the **debranching enzyme** (amylo-1,6-glucosidase) causes **Cori disease** or **Forbes disease** (Glycogen Storage Disease Type III). - This results in the accumulation of abnormally structured glycogen with short outer branches in the liver, muscle, and heart.

Q: What is the respiratory quotient of carbohydrates?

1. ***Option: 1 (Correct Answer)*** - The **respiratory quotient (RQ)** is the ratio of **carbon dioxide produced to oxygen consumed** during metabolism. - For carbohydrates, complete oxidation yields equal moles of CO2 and O2, resulting in an **RQ of 1.0**. - Example: C6H12O6 + 6O2 → 6CO2 + 6H2O, giving RQ = 6CO2/6O2 = 1.0 - This value reflects that carbohydrates are highly oxygenated molecules, requiring less external oxygen for their oxidation relative to the CO2 produced. *Option: 0.5* - An RQ of 0.5 is not observed for any major macronutrient during complete oxidation. - This value would imply significantly lower CO2 production relative to O2 consumption, which doesn't match any physiological substrate metabolism. *Option: 0.8* - An RQ of approximately 0.8 is characteristic of a **mixed diet** or the average value sometimes cited for **protein metabolism**. - Protein RQ typically ranges from 0.8-0.85, as proteins require more oxygen for their oxidation compared to the CO2 produced. - The exact RQ can vary depending on the specific amino acids being metabolized. *Option: 0.75* - An RQ around 0.7-0.75 may represent **fat-predominant metabolism** or a mixed diet with fats and carbohydrates. - Pure **fat metabolism** has an RQ of approximately **0.7**, as fats require substantial oxygen for oxidation due to their lower oxygen content relative to carbon and hydrogen. - Fats contain many C-H bonds and few C-O bonds, necessitating more oxygen for complete combustion.

Q: Most important carbohydrate store for maintaining blood glucose homeostasis -

Hepatic glycogen. ***Hepatic glycogen*** - The liver contains **100-120g of glycogen**, which is the most crucial carbohydrate store for **maintaining blood glucose homeostasis**. - **Hepatic glycogen** can be mobilized and released as glucose into the bloodstream to supply all body tissues, especially during fasting. - Although muscle glycogen is quantitatively larger (~400-500g), it cannot contribute to blood glucose as muscle lacks glucose-6-phosphatase. - The liver's unique ability to release free glucose makes hepatic glycogen the **most metabolically important** carbohydrate store. *Blood glucose* - **Blood glucose** (~5g total in circulation) represents carbohydrates available for immediate energy, not a storage form. - This is far too small to be considered a major carbohydrate reserve. *Glycogen in adipose tissue* - **Adipose tissue** primarily stores **fat (triglycerides)**, with negligible glycogen content. - Adipose tissue plays virtually no role in carbohydrate storage. *None of the options* - This is incorrect because **hepatic glycogen** is indeed the most important carbohydrate store for glucose homeostasis.

Question 1

What is the cause of lactose intolerance?

Accepted Answer

Deficiency of Lactase

Answer

Deficiency of Galactokinase

Answer

Deficiency of Uridyl transferase

Answer

Deficiency of Enteropeptidase

Question 2

Which of the following is an aldose sugar?

Accepted Answer

Glyceraldehyde

Answer

Ribulose

Answer

Fructose

Answer

None of the options

Question 3

An adolescent male patient presents to you with exercise intolerance. He gives a history of developing cramps on exertion. Which of the following enzyme deficiencies could be the cause?

Accepted Answer

Myophosphorylase

Answer

Hexokinase

Answer

Glucose-6-phosphatase

Answer

Hepatic glycogen phosphorylase

Question 4

When the insulin to glucagon ratio decreases, which enzyme is primarily active?

Accepted Answer

Glycogen phosphorylase

Answer

Glucose-6-phosphatase

Answer

Pyruvate carboxylase

Answer

Fructose 1,6-bisphosphatase

Question 5

Which one of the following shows allosteric inhibition of glycolysis?

Accepted Answer

Citrate

Answer

Amino acid alanine

Answer

2,3 BPG

Answer

Malonic acid

Question 6

Classical galactosemia (Type I) is due to deficiency of which enzyme?

Accepted Answer

Galactose-1-phosphate uridyltransferase

Answer

Fructose-1,6-bisphosphatase

Answer

Adenine phosphoribosyltransferase (APRT)

Answer

Hexokinase

Question 7

Glycemic index is defined as:

Accepted Answer

Measure of the change in the blood glucose following ingestion of carbohydrate.

Answer

Measure of the change in blood glucose following ingestion of protein.

Answer

Measure of the change in blood glucose following ingestion of fat.

Answer

Measure of glucose control over a period of time.

Question 8

Enzyme deficient in Hers disease -

Accepted Answer

Liver phosphorylase

Answer

Muscle phosphorylase

Answer

Acid maltase

Answer

Debranching enzyme

Question 9

What is the respiratory quotient of carbohydrates?

Accepted Answer

1

Answer

0.5

Answer

0.8

Answer

0.75

Question 10

Most important carbohydrate store for maintaining blood glucose homeostasis -

Accepted Answer

Hepatic glycogen

Answer

Blood glucose

Answer

Glycogen in adipose tissue

Answer

None of the options

Carbohydrate Metabolism — MCQs

Carbohydrate Metabolism — MCQs

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