Which metabolic pathway provides instant energy to muscles?
During starvation, muscle uses?
Which of the following organs does not primarily utilize fatty acids for energy?
Metabolic changes seen in starvation include all of the following except?
A normal female, whose father is color blind, marries a normal man. What are the chances of their son being color blind?
What does salvage purine synthesis refer to?
Which of the following organs does not primarily utilize the salvage pathway of purine nucleotide synthesis?
What is the end product of purine metabolism in humans?
Rate limiting step in pyrimidine synthesis?
Hereditary orotic aciduria Type-I is due to deficiency of?
NEET-PG 2013 - Biochemistry NEET-PG Practice Questions and MCQs
Question 61: Which metabolic pathway provides instant energy to muscles?
- A. Embden-Meyerhof pathway (Correct Answer)
- B. HMP shunt
- C. Cori cycle
- D. TCA cycle
Explanation: ***Embden-Meyerhof pathway*** - This pathway, also known as **glycolysis**, rapidly breaks down glucose into pyruvate to produce **ATP without oxygen**, providing instant energy to muscles during high-intensity activity. - Generates a net of **two ATP molecules** per glucose molecule, which is crucial for quick bursts of energy. *HMP shunt* - The **hexose monophosphate shunt** primarily produces **NADPH** for reductive biosynthesis and **ribose-5-phosphate** for nucleotide synthesis, not immediate large-scale ATP for muscle contraction. - Plays a role in protecting cells from **oxidative stress** and synthesizing precursors for DNA and RNA. *Cori cycle* - The **Cori cycle** involves the recycling of **lactate** produced in muscles back to glucose in the liver, which is a slower process for maintaining glucose homeostasis rather than providing instant muscle energy. - It helps prevent **lactic acidosis** during strenuous activity but is not a primary pathway for rapid ATP generation. *TCA cycle* - The **TCA cycle (Krebs cycle)** is part of **aerobic respiration** and produces a significant amount of ATP, but it is a slower, more sustained energy production pathway that requires oxygen. - Primarily active during **lower-intensity**, longer-duration activities when oxygen supply is adequate.
Question 62: During starvation, muscle uses?
- A. Fatty acids (Correct Answer)
- B. Ketone bodies
- C. Glucose
- D. Proteins
Explanation: ***Fatty acids*** - During **early and moderate starvation**, muscle tissue primarily uses **fatty acids** released from adipose tissue as its main energy source. - This preserves **glucose** for essential organs like the brain and red blood cells, which have an obligate need for it. *Ketone bodies* - While muscle can utilize **ketone bodies** during prolonged starvation, they are predominantly a fuel source for the **brain** once fatty acid stores are depleted. - The brain's adaptation to using ketones helps reduce the reliance on gluconeogenesis and preserves muscle protein. *Glucose* - Muscle primarily uses **glucose** as its main energy source in the fed state or during high-intensity exercise. - However, during starvation, muscle significantly reduces its glucose uptake to conserve it for other vital organs. *Proteins* - Muscle protein can be broken down into **amino acids** for gluconeogenesis in the liver to maintain blood glucose levels during prolonged starvation. - However, this is a **catabolic process** and not the primary preferred fuel source for muscle activity itself, as it leads to muscle wasting.
Question 63: Which of the following organs does not primarily utilize fatty acids for energy?
- A. Brain (Correct Answer)
- B. Muscle
- C. Liver
- D. Kidney
Explanation: ***Brain*** - The **brain primarily uses glucose** as its main energy source because fatty acids cannot efficiently cross the **blood-brain barrier**. - During prolonged starvation, the brain can adapt to use **ketone bodies**, which are derived from fatty acid breakdown in the liver. *Muscle* - **Skeletal muscle** can utilize both **glucose and fatty acids** for energy, with fatty acids becoming a more prominent fuel source during prolonged exercise and at rest. - **Cardiac muscle** (heart) heavily relies on **fatty acid oxidation** as its primary energy substrate, especially during basal conditions. *Liver* - The **liver is highly metabolically flexible** and readily oxidizes fatty acids for its own energy needs, particularly during fasting states. - It also plays a key role in **fatty acid metabolism**, including synthesis, breakdown, and conversion into ketone bodies. *Kidney* - The **renal cortex** is rich in mitochondria and has a high metabolic rate, primarily utilizing **fatty acid oxidation** to meet its significant energy demands for filtration and reabsorption. - While the renal medulla can use glucose, the cortex's reliance on fatty acids makes it a significant consumer.
Question 64: Metabolic changes seen in starvation include all of the following except?
- A. Ketogenesis
- B. Protein degradation
- C. Increased gluconeogenesis
- D. Increased glycolysis (Correct Answer)
Explanation: ***Increased glycolysis*** - In starvation, the body's primary goal is to conserve **glucose** for essential organs like the brain, as glucose supply is limited. Therefore, glycolysis, the breakdown of glucose, is *decreased*, not increased. - The body shifts to using alternative fuels such as **fatty acids** and **ketone bodies** to spare glucose. *Increased gluconeogenesis* - **Gluconeogenesis**, the synthesis of glucose from non-carbohydrate precursors like amino acids and glycerol, is *increased* during starvation to maintain blood glucose levels. - This process is crucial for providing glucose to tissues that primarily rely on it, such as the brain and red blood cells. *Ketogenesis* - **Ketogenesis**, the production of ketone bodies from fatty acids, is significantly *increased* during prolonged starvation. - **Ketone bodies** become a major energy source for the brain and other tissues when glucose is scarce, helping to spare muscle protein. *Protein degradation* - **Protein degradation** (proteolysis) is *increased* during starvation, especially in the initial phases, to provide amino acids for gluconeogenesis. - Muscle protein is a primary source of these amino acids, contributing to muscle wasting observed in prolonged starvation.
Question 65: A normal female, whose father is color blind, marries a normal man. What are the chances of their son being color blind?
- A. 25%
- B. 50% (Correct Answer)
- C. 75%
- D. No chance
Explanation: ***50%*** - The mother is a **carrier** because her father is colorblind, meaning she has one normal X chromosome and one affected X chromosome. - A son inherits his X chromosome from his mother; there is a **50% chance** that he will inherit the X chromosome carrying the colorblindness gene. *25%* - This percentage is typically associated with **autosomal recessive** inheritance patterns, not X-linked traits like colorblindness. - It would imply a different genetic setup for the parents than described, such as both parents being carriers for an autosomal recessive condition. *75%* - This probability would suggest a more complex genetic scenario or a condition with **incomplete penetrance** or a dominant inheritance pattern, which does not apply to X-linked recessive colorblindness in this context. - It does not align with the mendelian inheritance pattern for X-linked recessive traits when the mother is a carrier and the father is unaffected. *No chance* - This would only be true if the mother was **not a carrier** of the colorblindness gene. - Since her father was colorblind, she must have inherited his affected X chromosome, making her an obligate carrier.
Question 66: What does salvage purine synthesis refer to?
- A. Synthesis of purine nucleotides from purine bases (Correct Answer)
- B. Synthesis of purine nucleotides from ribose-5-phosphate
- C. Synthesis of purine nucleotides from simple precursors (de novo synthesis)
- D. Synthesis of purine nucleotides from degraded RNA
Explanation: ***Synthesis of purine nucleotides from purine bases*** - **Salvage pathways** recycle pre-existing purine or pyrimidine bases (from nucleic acid degradation) by re-attaching them to a **ribose phosphate** to form a new nucleotide. - This process is energy-efficient as it bypasses several steps of the de novo synthesis pathway, utilizing enzymes like **adenine phosphoribosyltransferase (APRT)** and **hypoxanthine-guanine phosphoribosyltransferase (HGPRT)**. *Synthesis of purine nucleotides from ribose-5-phosphate.* - While **ribose-5-phosphate** is a precursor, the complete synthesis from this molecule is part of the **de novo pathway**, which starts with PRPP (phosphoribosyl pyrophosphate) formation from ribose-5-phosphate. - This option does not specify the direct reuse of a pre-formed purine base, which is the hallmark of salvage. *Synthesis of purine nucleotides from simple precursors (de novo synthesis).* - **De novo synthesis** is the creation of nucleotides from scratch using simple metabolic precursors like amino acids (glycine, aspartate, glutamine), CO2, and THF derivatives. - This contrasts with salvage pathways, which recycle existing bases. *Synthesis of purine nucleotides from degraded RNA.* - Degraded RNA breaks down into **nucleotides**, which can then be further broken down into **purine bases** and ribose phosphates. - The direct synthesis of purine nucleotides from *degraded RNA* involves recovering the individual bases or nucleosides, then converting them to nucleotides via salvage, not directly using the entire degraded RNA.
Question 67: Which of the following organs does not primarily utilize the salvage pathway of purine nucleotide synthesis?
- A. RBC
- B. Leukocytes
- C. Liver (Correct Answer)
- D. Brain
Explanation: ***Liver*** - The **liver** is capable of both *de novo* synthesis and the salvage pathway of purine nucleotides, but it primarily utilizes the **de novo pathway** due to its high metabolic capacity and central role in biosynthesis for the entire body. - While salvage pathways exist, the liver's robust *de novo* synthesis allows it to readily produce purines from simple precursors, making it less reliant on salvaging pre-formed bases. *Brain* - The **brain** relies heavily on the **salvage pathway** for purine nucleotide synthesis because it has a limited capacity for *de novo* purine synthesis. - This dependency makes the brain particularly vulnerable to deficiencies in salvage enzymes, such as in **Lesch-Nyhan syndrome** where HGPRT deficiency leads to severe neurological dysfunction. *RBC* - **Red blood cells (RBCs)** are anucleated and lack the machinery for *de novo* purine synthesis, making them entirely dependent on the **salvage pathway** to maintain their purine nucleotide pool. - They salvage pre-formed purine bases and nucleosides from the plasma to synthesize necessary adenine and guanine nucleotides. *Leukocytes* - **Leukocytes**, particularly lymphocytes, have a high turn-over rate and metabolic activity, and they primarily rely on the **salvage pathway** for purine nucleotide synthesis. - The **immune system's rapid proliferation** and response demand efficient nucleotide synthesis, and the salvage pathway offers a quick and energy-efficient way to achieve this.
Question 68: What is the end product of purine metabolism in humans?
- A. Uric acid (Correct Answer)
- B. Carbon Dioxide
- C. Allantoin
- D. None of the options
Explanation: ***Uric acid*** - **Uric acid** is the final breakdown product of **purine metabolism** in humans. - It is formed from the degradation of **adenosine** and **guanosine**, with xanthine oxidase playing a key role in its synthesis. *Allantoin* - **Allantoin** is the end product of **purine metabolism** in most mammals other than primates, as they possess the enzyme **uricase** to further break down uric acid. - Humans lack **uricase**, hence allantoin is not the end product in humans. *Carbon Dioxide* - **Carbon dioxide** is a major end product of **carbohydrate** and **fat metabolism** through cellular respiration. - It is not directly associated with the degradation pathway of purines. *None of the options* - This option is incorrect because **uric acid** is indeed the definitive end product of purine metabolism in humans.
Question 69: Rate limiting step in pyrimidine synthesis?
- A. Aspartate transcarbamoylase (ATCase)
- B. Dihydroorotate dehydrogenase
- C. Dihydro-orotase
- D. Carbamoyl phosphate synthase-II (Correct Answer)
Explanation: ***Carbamoyl phosphate synthetase II (CPS-II)*** - **CPS-II** is the **committed and rate-limiting enzyme** in **de novo pyrimidine synthesis** in **mammals (including humans)** - It catalyzes the formation of **carbamoyl phosphate** from glutamine, CO₂, and 2 ATP in the **cytoplasm** - This is the **first committed step** and the main **regulatory checkpoint**, inhibited by UTP (feedback inhibition) and activated by PRPP and ATP - CPS-II is part of the **CAD complex** (carbamoyl phosphate synthetase, aspartate transcarbamoylase, dihydroorotase) in mammals *Aspartate transcarbamoylase (ATCase)* - ATCase catalyzes the **second step**: condensation of carbamoyl phosphate with aspartate to form carbamoyl aspartate - While ATCase is the **rate-limiting step in bacteria** (E. coli), in **mammals** it is part of the CAD complex and **not the primary regulatory step** - This option is incorrect for human/mammalian biochemistry tested in NEET PG *Dihydro-orotase* - The **third enzyme** in the pathway, converting carbamoyl aspartate to dihydroorotate - Part of the CAD complex in mammals but **not the rate-limiting step** *Dihydroorotate dehydrogenase* - Catalyzes the **fourth step**: oxidation of dihydroorotate to orotate - Located on the **outer surface of the inner mitochondrial membrane** (only mitochondrial enzyme in the pathway) - Important enzyme but **not rate-limiting**
Question 70: Hereditary orotic aciduria Type-I is due to deficiency of?
- A. Orotate phosphoribosyl transferase
- B. UMP synthase (Correct Answer)
- C. Orotic acid decarboxylase
- D. All of the options
Explanation: ***UMP synthase*** - Hereditary orotic aciduria Type-I is caused by a deficiency of the **bifunctional enzyme UMP synthase** (also called UMP synthase complex). - UMP synthase catalyzes two sequential reactions in the *de novo* pyrimidine synthesis pathway: 1. **OPRT activity**: Converts orotate → orotidine 5'-monophosphate (OMP) 2. **ODC activity**: Converts OMP → uridine 5'-monophosphate (UMP) - This is the **most precise and complete answer** as it identifies the actual enzyme complex that is deficient. - **Clinical features**: Megaloblastic anemia, growth retardation, immunodeficiency; responds to oral uridine supplementation. *Orotate phosphoribosyl transferase* - This represents only **one of the two catalytic activities** of the UMP synthase enzyme (the first step). - While this activity is indeed deficient in Type-I orotic aciduria, naming only this activity is **incomplete** because the enzyme has two functions. - This would be a **partial answer** rather than the complete enzyme name. *Orotic acid decarboxylase* - This represents only **the second catalytic activity** of the UMP synthase enzyme (converts OMP to UMP). - Like OPRT, this activity is also deficient, but naming only this component is **incomplete**. - **Type II orotic aciduria** (extremely rare) involves isolated ODC deficiency without OPRT deficiency. *All of the options* - While technically both OPRT and ODC activities are affected in Type-I orotic aciduria, the **standard nomenclature** refers to the deficient enzyme as **"UMP synthase"** - the name of the complete bifunctional enzyme. - In medical terminology and examination context, we identify enzyme deficiencies by the **name of the enzyme complex**, not by listing all its individual catalytic activities. - Therefore, **"UMP synthase"** is the single most accurate and complete answer.