Amino Acid Metabolism — MCQs

Amino Acid Metabolism Indian Medical PG Practice Questions and MCQs

Question 541: Coenzyme for phenylalanine hydroxylase is?

A. Tetrahydrofolate
B. Pyridoxal phosphate
C. S-adenosyl methionine
D. Tetrahydrobiopterin (Correct Answer)

Explanation: ***Tetrahydrobiopterin*** - **Tetrahydrobiopterin (BH4)** is an essential coenzyme for aromatic amino acid hydroxylases, including **phenylalanine hydroxylase (PAH)**. - PAH converts **phenylalanine** to **tyrosine**, and deficiencies in BH4 or PAH itself lead to *phenylketonuria (PKU)*. *Tetrahydrofolate* - **Tetrahydrofolate (THF)** is a coenzyme derived from **folic acid** and is primarily involved in **one-carbon metabolism**, including **purine** and **pyrimidine synthesis**, and various amino acid interconversions. - It does not directly act as a coenzyme for phenylalanine hydroxylase. *Pyridoxal phosphate* - **Pyridoxal phosphate (PLP)**, a derivative of **vitamin B6**, is a crucial coenzyme for many enzymes involved in **amino acid metabolism**, particularly in **transamination**, **decarboxylation**, and side-chain cleavage reactions. - It is not the coenzyme for phenylalanine hydroxylase. *S-adenosyl methionine* - **S-adenosyl methionine (SAM)** is a major **methyl donor** in various biochemical reactions, important for the synthesis of **neurotransmitters**, **hormones**, and **phospholipids**. - While essential for many metabolic pathways, it is not involved as a coenzyme for phenylalanine hydroxylase.

Question 542: Which of the following is not a metabolic product of the urea cycle?

A. Citrulline
B. Arginine
C. Alanine (Correct Answer)
D. Ornithine

Explanation: ***Alanine*** - **Alanine** is an amino acid primarily involved in the **glucose-alanine cycle** for glucose production and ammonia transport, not as a direct metabolic product within the urea cycle. - While it plays a role in nitrogen metabolism, it is not synthesized or directly consumed as an intermediate in the reactions that convert ammonia to urea. *Citrulline* - **Citrulline** is a key intermediate formed during the second step of the urea cycle when **ornithine carbamoyltransferase** combines carbamoyl phosphate with ornithine. - It is then transported out of the mitochondrion into the cytosol to continue the cycle. *Ornithine* - **Ornithine** is an amino acid that acts as a **catalytic intermediate** in the urea cycle, being regenerated at the end of the cycle to combine with carbamoyl phosphate. - It does not directly contribute a nitrogen atom to urea but is essential for the cycle's continuation. *Arginine* - **Arginine** is an amino acid that is a direct precursor to urea in the penultimate step of the urea cycle, where **arginase** cleaves it into urea and ornithine. - It provides one of the nitrogen atoms and the carbon atom for the formation of urea.

Question 543: Which coenzyme is not required in the formation of glutamate?

A. None of the above
B. Pyridoxal phosphate
C. Thiamine pyrophosphate (Correct Answer)
D. Niacin

Explanation: ***Thiamine pyrophosphate*** - **Thiamine pyrophosphate (TPP)** is a coenzyme derived from **vitamin B1** that is essential for reactions involving decarboxylation, such as those catalyzed by pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase. - The formation of glutamate primarily involves transamination or reductive amination, which do not require TPP. *Pyridoxal phosphate* - **Pyridoxal phosphate (PLP)**, derived from **vitamin B6**, is a crucial coenzyme for **transamination reactions**, which are a major pathway for glutamate synthesis (e.g., from alpha-ketoglutarate). - It also plays a role in decarboxylation and deamination reactions of amino acids. *Niacin* - **Niacin (vitamin B3)** is a precursor for **NAD+** and **NADP+**, which are essential coenzymes in many metabolic pathways. - **NADPH**, derived from NADP+, is required as a reductant in the **reductive amination** of **alpha-ketoglutarate** to form glutamate, catalyzed by glutamate dehydrogenase. *None of the above* - This option is incorrect because **thiamine pyrophosphate** is indeed not required for the formation of glutamate. - The other two coenzymes, **pyridoxal phosphate** and **niacin (as NAD(P)H)**, are involved in glutamate synthesis.

Question 544: Fumarate is formed from which amino acid?

A. Methionine
B. Valine
C. Histidine
D. Tyrosine (Correct Answer)

Explanation: ***Tyrosine*** - **Tyrosine** is a **glucogenic and ketogenic amino acid** that is catabolized to acetoacetate and fumarate. - **Fumarate** then enters the **citric acid cycle (Krebs cycle)**, whereas acetoacetate is a ketone body. *Methionine* - **Methionine** is an **essential amino acid** and a precursor for **S-adenosylmethionine (SAM)**, a methyl donor in many reactions. - Its catabolism produces **succinyl CoA**, not fumarate, through a series of steps via propionyl CoA. *Valine* - **Valine** is a **branched-chain amino acid (BCAA)** that is exclusively **glucogenic**. - Its catabolism ultimately leads to the formation of **succinyl CoA**, which can enter the citric acid cycle. *Histidine* - **Histidine** is an **essential amino acid** that is catabolized to **formiminoglutamate (FIGLU)**. - FIGLU is then converted to **glutamate**, which can eventually be deaminated to α-ketoglutarate, a citric acid cycle intermediate, but not directly fumarate.

Question 545: In phenylketonuria, which substance should be restricted in the diet?

A. Tyrosine
B. Phenylalanine (Correct Answer)
C. Maize
D. None of the options

Explanation: ***Phenylalanine*** - **Phenylketonuria (PKU)** is a genetic disorder where the body cannot effectively metabolize **phenylalanine** due to a deficiency in the enzyme **phenylalanine hydroxylase**. - Restricting dietary phenylalanine is crucial to prevent the accumulation of toxic byproducts that can lead to severe neurological damage and developmental delays. *Tyrosine* - Tyrosine is normally synthesized from phenylalanine. In PKU, this conversion is impaired. - While phenylalanine must be restricted, tyrosine supplementation is often necessary for individuals with PKU, as it becomes a conditionally essential amino acid. *Maize* - Maize (corn) is a carbohydrate-rich food and does not contain high levels of phenylalanine that would necessitate its restriction in PKU. - Dietary management in PKU focuses on regulating protein intake, as phenylalanine is an amino acid found in proteins. *None of the options* - This option is incorrect because phenylalanine must be strictly restricted in the diet of individuals with phenylketonuria to manage the condition effectively. - Without dietary restriction, the accumulation of phenylalanine can lead to severe and irreversible neurological damage.

Question 546: Which of the following is classified as an essential amino acid?

A. Aspartic acid
B. Glutamic acid
C. Lysine (Correct Answer)
D. None of the options

Explanation: ***Lysine*** - **Essential amino acids** cannot be synthesized by the human body and must be obtained through the diet. - Lysine is one of the **nine essential amino acids** necessary for **protein synthesis**, collagen formation, calcium absorption, and other metabolic functions. - Remembered by the mnemonic: **PVT TIM HALL** (Phe, Val, Thr, Trp, Ile, Met, His, Arg*, Leu, Lys). *Aspartic acid* - Aspartic acid is a **non-essential amino acid**, meaning the body can synthesize it from oxaloacetate through transamination. - It plays a role in the **urea cycle** and **gluconeogenesis**. - Functions as an excitatory neurotransmitter in the central nervous system. *Glutamic acid* - Glutamic acid is also a **non-essential amino acid** that the body can readily produce from α-ketoglutarate. - It is a crucial excitatory **neurotransmitter** and a precursor for other amino acids like **glutamine** and **proline**. - Important in nitrogen metabolism and the urea cycle. *None of the options* - This option is incorrect because **lysine** is clearly an essential amino acid that must be obtained from dietary sources.

Question 547: Which of the following compounds is not formed with the involvement of glycine?

A. Purines
B. Glutathione
C. Thyroxine (Correct Answer)
D. Heme

Explanation: ***Thyroxine*** - **Thyroxine** (and other thyroid hormones) are derived from the amino acid **tyrosine**. - Their synthesis involves iodination and coupling reactions of tyrosine residues within the protein **thyroglobulin**. *Heme* - **Glycine** is a direct precursor for the initial step in **heme synthesis**. - It condenses with **succinyl CoA** to form α-amino-β-ketoadipate, which then decarboxylates to form δ-aminolevulinate (ALA). *Purines* - **Glycine** contributes part of its structure to the **purine ring**. - Specifically, the **nitrogen at position 7** and the **carbons at positions 4 and 5** of the purine ring are derived from glycine. *Glutathione* - **Glutathione** is a tripeptide composed of three amino acids: **glutamate**, **cysteine**, and **glycine**. - **Glycine** is the C-terminal amino acid of glutathione and is essential for its structure and function as an antioxidant.

Question 548: Which of the following statements about Maple Syrup Urine Disease (MSUD) is true?

A. FeCl3 turns navy blue
B. Asymptomatic
C. Deficiency of branched chain amino acid enzymes (Correct Answer)
D. Hyperaminoaciduria of aromatic amino acids

Explanation: ***Deficiency of branched chain amino acid enzymes*** - MSUD is caused by a deficiency in the **branched-chain alpha-keto acid dehydrogenase complex (BCKDC)**, an enzyme responsible for metabolizing **leucine, isoleucine, and valine**. - This enzyme deficiency leads to the accumulation of these **branched-chain amino acids (BCAAs)** and their corresponding branched-chain alpha-keto acids in the body. *Asymptomatic* - MSUD is a severe metabolic disorder that is **not asymptomatic**; it typically presents with distinct neurological and metabolic symptoms shortly after birth. - Clinical manifestations can include **poor feeding, lethargy, seizures**, and a characteristic **maple syrup odor** in urine and earwax. *FeCl3 turns navy blue* - The **ferric chloride test (FeCl3 test)** in MSUD typically yields a **green-gray or dark-green color** when testing for the accumulation of alpha-keto acids (specifically alpha-ketoisocaproic acid). - A **navy blue color** with FeCl3 is more characteristic of **alkaptonuria** due to the presence of homogentisic acid. *Hyperaminoaciduria of aromatic amino acids* - MSUD is characterized by the accumulation and excretion of **branched-chain amino acids (leucine, isoleucine, valine)** and their corresponding keto acids. - **Hyperaminoaciduria of aromatic amino acids** (e.g., phenylalanine, tyrosine, tryptophan) is seen in other conditions like **phenylketonuria (PKU)**, not MSUD.

Question 549: Serotonin is derived from -

A. Tyrosine
B. Tryptophan (Correct Answer)
C. Phenylalanine
D. Methionine

Explanation: ***Tryptophan*** - **Serotonin**, also known as 5-hydroxytryptamine (5-HT), is synthesized from the essential amino acid **tryptophan** through a two-step enzymatic pathway. - Tryptophan is first hydroxylated by tryptophan hydroxylase to 5-hydroxytryptophan (5-HTP), which is then decarboxylated by L-amino acid decarboxylase to form serotonin. *Tyrosine* - **Tyrosine** is a precursor for the synthesis of **catecholamines** (dopamine, norepinephrine, and epinephrine) and thyroid hormones. - It is not involved in the synthesis pathway for serotonin. *Phenylalanine* - **Phenylalanine** is an essential amino acid that is hydroxylated to form **tyrosine**. - Therefore, it is indirectly involved in catecholamine synthesis but not in serotonin synthesis. *Methionine* - **Methionine** is an essential amino acid primarily known for its role in protein synthesis and as a precursor for **S-adenosylmethionine (SAM)**, a methyl group donor in many biological reactions. - It does not serve as a direct precursor for serotonin.

Question 550: Catecholamines are synthesized from?

A. Tyrosine (Correct Answer)
B. Histidine
C. Methionine
D. Tryptophan

Explanation: ***Tyrosine*** - **Tyrosine** is the direct precursor amino acid for the synthesis of all **catecholamines**, including **dopamine**, **norepinephrine**, and **epinephrine**. - The synthesis pathway begins with the conversion of tyrosine to **L-DOPA** by tyrosine hydroxylase, followed by subsequent enzymatic steps. *Methionine* - **Methionine** is an essential amino acid primarily involved in **protein synthesis** and as a precursor for S-adenosylmethionine (SAM), a key methyl donor in various metabolic reactions. - It is not a direct precursor for the synthesis of **catecholamines**. *Histidine* - **Histidine** is the precursor for the synthesis of **histamine**, a neurotransmitter and inflammatory mediator. - It is not involved in the biosynthesis pathway of **catecholamines**. *Tryptophan* - **Tryptophan** is the precursor for the synthesis of **serotonin** and **melatonin**, important neurotransmitters and hormones. - It does not play a role in the synthesis of **catecholamines**.

Practice by Chapter

Protein Digestion and Absorption

Practice Questions

Transamination and Deamination

Practice Questions

Urea Cycle

Practice Questions

Disorders of Urea Cycle

Practice Questions

Metabolism of Individual Amino Acids

Practice Questions

Inborn Errors of Amino Acid Metabolism

Practice Questions

Phenylketonuria and Alkaptonuria

Practice Questions

Homocystinuria and Methionine Metabolism

Practice Questions

Synthesis of Biologically Important Compounds from Amino Acids

Practice Questions

Nitrogen Balance

Practice Questions

Ammonia Metabolism and Toxicity

Practice Questions

One-Carbon Transfer Reactions

Practice Questions

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