NEET-PG 2012 - Biochemistry Practice Questions

Q: Neonatal tyrosinemia is due to deficiency of which enzyme?

Hydroxyphenyl pyruvate hydroxylase. ***Hydroxyphenyl pyruvate hydroxylase*** - **Neonatal (transient) tyrosinemia** is caused by delayed maturation or deficiency of **hydroxyphenylpyruvate hydroxylase** (also called 4-hydroxyphenylpyruvate dioxygenase or HPPD). - This enzyme converts 4-hydroxyphenylpyruvate to homogentisic acid in tyrosine catabolism. - Common in **premature infants** and newborns, leading to elevated tyrosine levels in blood. - The condition is **benign and self-limiting**, usually resolving with **vitamin C supplementation** or as the enzyme matures. - Note: Severe hereditary deficiency of this enzyme causes **tyrosinemia type III**, a distinct and rare disorder. *Fumarylacetoacetate hydroxylase* - Deficiency of **fumarylacetoacetate hydroxylase (FAH)** causes **tyrosinemia type I** (hepatorenal tyrosinemia), NOT neonatal tyrosinemia. - This is a severe hereditary disorder with liver failure, renal tubular dysfunction, and accumulation of toxic metabolites like succinylacetone. - Distinct from the benign transient neonatal form. *Tyrosine transaminase* - Deficiency of **tyrosine transaminase** (tyrosine aminotransferase) causes **tyrosinemia type II** (Richner-Hanhart syndrome). - Presents with corneal ulcers, palmoplantar hyperkeratosis, and sometimes intellectual disability. *Tyrosinase* - Deficiency of **tyrosinase** causes **albinism**, characterized by lack of melanin pigment in skin, hair, and eyes. - Not involved in tyrosine catabolism but in melanin synthesis.

Q: Which enzyme is deficient in Isovaleric acidemia?

Isovaleryl CoA dehydrogenase. ***Isovaleryl CoA dehydrogenase*** - **Isovaleric acidemia** is an **autosomal recessive** metabolic disorder caused by a deficiency in the enzyme **isovaleryl-CoA dehydrogenase** - This enzyme is crucial for the metabolism of **leucine**, a branched-chain amino acid, leading to the accumulation of toxic byproducts like **isovaleryl-CoA** and **isovaleric acid** - Characteristic **sweaty feet odor** due to isovaleric acid accumulation *Phenylalanine hydroxylase* - A deficiency in **phenylalanine hydroxylase** is responsible for **phenylketonuria (PKU)**, a different metabolic disorder involving the metabolism of **phenylalanine** - This enzyme converts **phenylalanine to tyrosine**, and its deficiency leads to the accumulation of phenylalanine and its metabolites, causing neurological damage if untreated *Arginase* - A deficiency in **arginase** causes **argininemia (hyperargininemia)**, which is a disorder of the **urea cycle** - This enzyme converts **arginine into urea and ornithine**, and its deficiency leads to the buildup of arginine and ammonia in the blood, causing neurological symptoms and developmental delay *Methylmalonyl CoA mutase* - A deficiency in **methylmalonyl CoA mutase** causes **methylmalonic acidemia**, another organic acidemia distinct from isovaleric acidemia - This disorder involves **propionate metabolism** and can present with metabolic acidosis, but affects a different metabolic pathway than leucine catabolism

Q: Why is the citric acid cycle called an amphibolic pathway?

Metabolites are utilized in other pathways.. ***Metabolites are utilized in other pathways.*** - The citric acid cycle is termed **amphibolic** because it serves both catabolic (breakdown) and anabolic (synthetic) functions. - Its intermediates are constantly drawn off for biosynthesis of molecules like **amino acids**, **heme**, and **glucose**, meaning it's not solely degradative. *Both exergonic and endergonic reactions take place* - While both types of reactions do occur in many metabolic pathways, this is a general characteristic of metabolism and not specific to the definition of an **amphibolic pathway**. - The amphibolic nature specifically refers to the dual role in both **catabolism** and **anabolism**. *It can proceed in both forward and backward directions.* - This statement typically describes a **reversible pathway** or individual reversible reactions, not necessarily an amphibolic pathway. - The citric acid cycle is primarily an oxidative cycle that proceeds in a forward, cyclic direction under aerobic conditions. *The same enzymes can be used in reverse directions.* - While some individual enzymes within metabolic pathways can catalyze reversible reactions, this is not the defining characteristic of an **amphibolic pathway**. - The amphibolic designation refers to the overall pathway's contribution to both breakdown and synthesis of molecules.

Q: Lipogenesis is stimulated by?

Insulin. ***Insulin*** - **Insulin** is a key anabolic hormone that promotes the synthesis and storage of fat (lipogenesis) by increasing the uptake of glucose into adipose tissue and stimulating enzymes involved in fatty acid synthesis. - It enhances the conversion of excess carbohydrates into **triglycerides** for storage. *Glucagon* - **Glucagon** is a catabolic hormone that primarily promotes the breakdown of glycogen (glycogenolysis) and fat (lipolysis) to release glucose and fatty acids into the bloodstream, especially during fasting. - It generally **inhibits** lipogenesis and stimulates **gluconeogenesis**. *Epinephrine* - **Epinephrine** (adrenaline) is a stress hormone that promotes the breakdown of fat (lipolysis) to provide energy during acute stress or exercise. - It would **inhibit** lipogenesis, as its primary role is to mobilize energy stores. *Corticosteroids* - While **corticosteroids** can influence fat metabolism, their effect on lipogenesis is complex and often indirect. High levels can lead to fat redistribution (e.g., central obesity) rather than direct stimulation of overall lipogenesis. - Corticosteroids generally promote **lipolysis** in the extremities and can contribute to insulin resistance, which would hinder lipogenesis in some tissues.

Q: In which condition is the utilization of pyruvate in tissues decreased?

Beriberi. ***Beriberi*** - Beriberi is caused by **thiamine (vitamin B1) deficiency**, which is a crucial cofactor for the **pyruvate dehydrogenase complex (PDH)**. - A dysfunctional PDH enzyme leads to a decreased conversion of **pyruvate to acetyl-CoA**, thus **decreasing pyruvate utilization** and causing its accumulation. *Pernicious anemia* - This condition is caused by a deficiency in **vitamin B12 (cobalamin)**, typically due to a lack of intrinsic factor, leading to **megaloblastic anemia**. - While vitamin B12 is essential for various metabolic pathways, it does not directly impair the utilization of **pyruvate** by PDH. *Scurvy* - Scurvy results from **vitamin C (ascorbic acid) deficiency**, which is essential for collagen synthesis and acts as an antioxidant. - Vitamin C deficiency does not directly impact the activity of the **pyruvate dehydrogenase complex** or the utilization of pyruvate. *Pellagra* - Pellagra is caused by a deficiency in **niacin (vitamin B3)**, or its precursor, tryptophan. - Niacin is a component of **NAD+ and NADP+**, which are crucial coenzymes in many metabolic reactions, but its deficiency does not primarily manifest as decreased **pyruvate utilization**.

Q: Which of the following is not a component of a nucleotide?

Fatty acid. ***Fatty acid*** - A **fatty acid** is a component of **lipids**, such as triglycerides and phospholipids, which are structurally and functionally distinct from **nucleotides**. - **Nucleotides** are the building blocks of nucleic acids (DNA and RNA), whereas fatty acids are essential for cell membranes and energy storage. *Sugar* - A **pentose sugar** (either **deoxyribose** in DNA or **ribose** in RNA) is a fundamental component of every nucleotide. - This sugar forms the backbone of the nucleic acid strand, covalently linked to the phosphate group and the nitrogenous base. *Phosphate* - A **phosphate group** is a crucial component of a nucleotide, providing the negative charge and forming the phosphodiester bonds that link nucleotides together into a nucleic acid chain. - The number of phosphate groups (mono-, di-, or triphosphate) determines the nucleotide's energy state and function. *Base* - A **nitrogenous base** (adenine, guanine, cytosine, thymine, or uracil) is an essential component of a nucleotide, responsible for genetic information storage and pairing. - This base is attached to the pentose sugar and determines the specific identity of the nucleotide within the DNA or RNA sequence.

Q: The Central Dogma of molecular biology includes all except?

Reverse transcription. ***Reverse transcription*** - The **original Central Dogma** as proposed by Francis Crick described the unidirectional flow of genetic information: **DNA → RNA → Protein**. - **Reverse transcription** (RNA → DNA) was **not part of the original Central Dogma** and was only discovered later with the identification of **reverse transcriptase enzyme** in retroviruses by Baltimore and Temin (1970). - While reverse transcription is now recognized as an important biological process, it represents an **exception to the classical Central Dogma** rather than a core component. *Transcription* - **Transcription** (DNA → RNA) is a **fundamental process** within the Central Dogma. - It involves synthesis of RNA from a DNA template and is the first step in gene expression in all living organisms. *Translation* - **Translation** (RNA → Protein) is the **second major step** of the Central Dogma. - This process occurs on ribosomes where mRNA is decoded into a specific sequence of amino acids to form proteins. *RNA replication* - **RNA replication** (RNA → RNA), while primarily seen in RNA viruses, is considered part of the **expanded Central Dogma**. - It represents one of the possible flows of genetic information in biological systems, though not universal to all organisms.

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

Glyceraldehyde-3-phosphate dehydrogenase. ***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.

Q: Which enzyme is required for cutting the DNA strand during synthesis?

Topoisomerase. ***Topoisomerase*** - **Topoisomerases** are enzymes essential for DNA replication; they induce temporary **single- or double-strand breaks** in DNA to relieve **supercoiling** ahead of the replication fork. - This cutting and rejoining activity prevents the DNA from becoming excessively tangled and facilitates the unwinding process required for synthesis. *DNA polymerase* - **DNA polymerase** is responsible for **synthesizing new DNA strands** by adding nucleotides, not for cutting the DNA backbone. - It works by moving along the template strand, reading the bases, and then adding complementary nucleotides to the growing DNA strand. *DNA ligase* - **DNA ligase** functions to **join DNA fragments** together by forming phosphodiester bonds, especially in sealing Okazaki fragments during lagging strand synthesis. - Its role is to ligate (join) cut strands, not to initiate cuts in the DNA. *Helicase* - **Helicase** unwinds the DNA double helix into single strands using ATP hydrolysis; it **separates the two strands** but does not cut the phosphodiester backbone. - This enzyme creates the replication fork by disrupting hydrogen bonds between base pairs, making the DNA accessible for replication machinery.

Q: DNA microarrays allow detection of gene mutations through which process?

Hybridization. ***Hybridization*** - DNA microarrays detect mutations by **hybridizing labeled patient DNA** to **thousands of oligonucleotide probes** containing known DNA sequences immobilized on a solid surface. - A mismatch between the patient's DNA and the probe results in **reduced or absent hybridization**, indicating a **mutation or genetic variation**. - This principle allows **high-throughput screening** of multiple genes simultaneously. *Polymerase Chain Reaction* - PCR is used to **amplify specific DNA sequences exponentially**, creating millions of copies from minimal starting material. - While PCR may be used to **prepare DNA samples** before microarray analysis, it is **not the detection mechanism** on the chip itself. *Cloning* - Cloning involves creating **identical copies of DNA fragments, cells, or organisms** using vectors and host cells. - It's a method for **producing large quantities** of specific DNA sequences but **not a detection technique** for mutations on microarrays. *Southern Blotting* - Southern blotting detects specific DNA sequences through **gel electrophoresis, membrane transfer, and probe hybridization**. - While it also uses hybridization, it is a **low-throughput technique** analyzing one sample at a time, unlike the **high-throughput parallel analysis** of DNA microarrays.

Question 1

Neonatal tyrosinemia is due to deficiency of which enzyme?

Accepted Answer

Hydroxyphenyl pyruvate hydroxylase

Answer

Tyrosine transaminase

Answer

Fumarylacetoacetate hydroxylase

Answer

Tyrosinase

Question 2

Which enzyme is deficient in Isovaleric acidemia?

Accepted Answer

Isovaleryl CoA dehydrogenase

Answer

Phenylalanine hydroxylase

Answer

Arginase

Answer

Methylmalonyl CoA mutase

Question 3

Why is the citric acid cycle called an amphibolic pathway?

Accepted Answer

Metabolites are utilized in other pathways.

Answer

Both exergonic and endergonic reactions take place

Answer

It can proceed in both forward and backward directions.

Answer

The same enzymes can be used in reverse directions.

Question 4

Lipogenesis is stimulated by?

Accepted Answer

Insulin

Answer

Glucagon

Answer

Epinephrine

Answer

Corticosteroids

Question 5

In which condition is the utilization of pyruvate in tissues decreased?

Accepted Answer

Beriberi

Answer

Pernicious anemia

Answer

Scurvy

Answer

Pellagra

Question 6

Which of the following is not a component of a nucleotide?

Accepted Answer

Fatty acid

Answer

Sugar

Answer

Base

Answer

Phosphate

Question 7

The Central Dogma of molecular biology includes all except?

Accepted Answer

Reverse transcription

Answer

Transcription

Answer

RNA replication

Answer

Translation

Question 8

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

Accepted Answer

Glyceraldehyde-3-phosphate dehydrogenase

Answer

Pyruvate kinase

Answer

Enolase

Answer

PFK-1

Question 9

Which enzyme is required for cutting the DNA strand during synthesis?

Accepted Answer

Topoisomerase

Answer

DNA polymerase

Answer

DNA ligase

Answer

Helicase

Question 10

DNA microarrays allow detection of gene mutations through which process?

Accepted Answer

Hybridization

Answer

Polymerase Chain Reaction

Answer

Cloning

Answer

Southern Blotting

NEET-PG 2012 — Biochemistry