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Questions and Answers
Patient A suffers from McArdle disease.
Patient A suffers from McArdle disease.
True (A)
Why does Patient A experience muscle pain and extreme tiredness after exercise?
Why does Patient A experience muscle pain and extreme tiredness after exercise?
Due to muscle glycogen phosphorylase deficiency, Patient A cannot effectively break down glycogen for energy during exercise, leading to pain and tiredness.
Why was blood lactate concentration increased in the control patient but not in Patient A?
Why was blood lactate concentration increased in the control patient but not in Patient A?
The control patient can utilize glycogen for anaerobic metabolism, producing lactate, while Patient A cannot due to the deficiency.
What is the expected level of muscle glycogen in Patient A after taking a meal and testing 4 hours later?
What is the expected level of muscle glycogen in Patient A after taking a meal and testing 4 hours later?
How would glucose supplements help alleviate Patient A's discomfort during and after exercise?
How would glucose supplements help alleviate Patient A's discomfort during and after exercise?
How are higher amounts of triglycerides beneficial for a migrating bird?
How are higher amounts of triglycerides beneficial for a migrating bird?
How many molecules of ATP will be produced through complete beta-oxidation of 1 molecule of lignoceric acid (24 carbons)?
How many molecules of ATP will be produced through complete beta-oxidation of 1 molecule of lignoceric acid (24 carbons)?
Identify the processes that lead to the formation of reactive oxygen species.
Identify the processes that lead to the formation of reactive oxygen species.
What is the role of Vitamin E as an antioxidant?
What is the role of Vitamin E as an antioxidant?
What is the role of Vitamin C in the prevention of lipid peroxidation?
What is the role of Vitamin C in the prevention of lipid peroxidation?
Why does the build-up of ammonium ion in the blood harm neural cells?
Why does the build-up of ammonium ion in the blood harm neural cells?
Which urea cycle enzyme(s) might be defective in Infant A with low blood urea nitrogen?
Which urea cycle enzyme(s) might be defective in Infant A with low blood urea nitrogen?
Which enzyme deficiency might be present in Infant B with elevated plasma glutamine?
Which enzyme deficiency might be present in Infant B with elevated plasma glutamine?
Identify the metabolic pathways A, B, and C in Figure 6.
Identify the metabolic pathways A, B, and C in Figure 6.
Will metabolic pathways B and C be functional in a type 1 diabetic individual?
Will metabolic pathways B and C be functional in a type 1 diabetic individual?
Why is the brain able to use ketone bodies under starvation conditions?
Why is the brain able to use ketone bodies under starvation conditions?
Which of the following statements about the reaction in its forward direction is FALSE?
Which of the following statements about the reaction in its forward direction is FALSE?
Krebs cycle is different from glycolysis as it ____________________________.
Krebs cycle is different from glycolysis as it ____________________________.
Oxidative phosphorylation differs from substrate-level phosphorylation as it____________________________.
Oxidative phosphorylation differs from substrate-level phosphorylation as it____________________________.
The coupled reaction is best described as a/an ________________ reaction.
The coupled reaction is best described as a/an ________________ reaction.
Which of the following statements about ATP in human cells is CORRECT?
Which of the following statements about ATP in human cells is CORRECT?
A hexokinase enzyme is treated with concentrated hydrochloric acid. Which of the following are least likely to be affected by this treatment?
A hexokinase enzyme is treated with concentrated hydrochloric acid. Which of the following are least likely to be affected by this treatment?
Which of the following statements about hydrophobic interactions in protein folding is FALSE?
Which of the following statements about hydrophobic interactions in protein folding is FALSE?
Enzymes that catalyse removal of hydrogen atoms from one substrate and addition of hydrogen atoms to another substrate are termed as _________.
Enzymes that catalyse removal of hydrogen atoms from one substrate and addition of hydrogen atoms to another substrate are termed as _________.
In spectrophotometry, the absorbance of the analyte is_________________.
In spectrophotometry, the absorbance of the analyte is_________________.
When using a standard curve for determining the concentration of a substance, the standard curve is NOT usable when ______________________.
When using a standard curve for determining the concentration of a substance, the standard curve is NOT usable when ______________________.
The glycogen in muscle does not contribute to the maintenance of blood glucose level as the muscle does not express the enzyme _________________.
The glycogen in muscle does not contribute to the maintenance of blood glucose level as the muscle does not express the enzyme _________________.
Which of the following CORRECTLY describes the action of insulin in regulation of blood glucose levels?
Which of the following CORRECTLY describes the action of insulin in regulation of blood glucose levels?
Which of the following statements about blood glucose regulation is FALSE?
Which of the following statements about blood glucose regulation is FALSE?
In fatty acid synthesis, _________________ is the active donor of ___________ carbon atoms to elongate the growing fatty acid chain.
In fatty acid synthesis, _________________ is the active donor of ___________ carbon atoms to elongate the growing fatty acid chain.
The gene that codes for liver aldehyde dehydrogenase is mutated in an individual. Which of the following will most likely happen during alcohol consumption by the individual?
The gene that codes for liver aldehyde dehydrogenase is mutated in an individual. Which of the following will most likely happen during alcohol consumption by the individual?
In ammonia metabolism, α-ketoglutarate is produced from glutamate in the liver through _______________________
In ammonia metabolism, α-ketoglutarate is produced from glutamate in the liver through _______________________
In the above redox reaction, which is an oxidizing agent?
In the above redox reaction, which is an oxidizing agent?
The most likely process occurring when a badminton player undergoes training for 2-hours is __________________.
The most likely process occurring when a badminton player undergoes training for 2-hours is __________________.
At substrate concentration of 2.0mM and in the absence of inhibitor, all the enzyme active sites are occupied by substrate molecules.
At substrate concentration of 2.0mM and in the absence of inhibitor, all the enzyme active sites are occupied by substrate molecules.
From the data, a Michaelis-Menten curve can be plotted and the maximum velocity of both reactions can be determined.
From the data, a Michaelis-Menten curve can be plotted and the maximum velocity of both reactions can be determined.
The inhibitor is likely to be a competitive inhibitor.
The inhibitor is likely to be a competitive inhibitor.
In both reactions, product formation increases as the substrate concentration increases from 0.2 to 1.2 mM.
In both reactions, product formation increases as the substrate concentration increases from 0.2 to 1.2 mM.
The reaction without inhibitor produces more products than the reaction with inhibitor at any given substrate concentration.
The reaction without inhibitor produces more products than the reaction with inhibitor at any given substrate concentration.
Study Notes
McArdle Disease
- Patient A suffers from McArdle disease, a condition caused by a deficiency in muscle glycogen phosphorylase.
- This deficiency prevents the breakdown of glycogen into glucose, which is necessary for energy production during exercise.
Muscle Pain and Tiredness
- Patient A experiences muscle pain and tiredness because his muscles cannot access stored glycogen for energy.
- This leads to insufficient ATP production, causing muscle fatigue and pain.
Blood Lactate Levels
- The Control patient shows an increased blood lactate concentration after exercise because their muscles can break down glycogen, leading to glycolysis and the production of lactate.
- Patient A's blood lactate levels remain low after exercise because his muscles cannot utilize glycogen for energy, resulting in a lack of lactate production from glycolysis.
Muscle Glycogen Levels
- Patient A's muscle glycogen levels are likely to be normal four hours after a meal because he can still store glycogen despite his inability to break it down effectively.
Glucose Supplements
- Consuming glucose supplements during exercise helps alleviate Patient A's discomfort because it provides an external source of glucose, bypassing his deficient glycogen breakdown pathway.
- This allows for increased ATP production and energy for muscle activity.
Fuel Sources For Long-Distance Migration
- Triglycerides are a beneficial fuel source for long-distance migration in birds because they are energy-dense and can be stored in large amounts.
- Glycogen is a less energy-dense fuel source.
Beta Oxidation of Lignoceric Acid
- Lignoceric acid is a saturated fatty acid with 24 carbon atoms per molecule.
- The complete beta-oxidation of lignoceric acid yields 11 molecules of acetyl-CoA, 10 molecules of NADH, and 10 molecules of FADH2.
Reactive Oxygen Species (ROS) Formation
- The formation of ROS can occur during normal metabolic processes, such as oxidative phosphorylation in the mitochondria.
- ROS can also be produced by exogenous factors, such as exposure to pollutants or radiation.
Role of Antioxidants in Preventing Lipid Peroxidation
- Vitamin E acts as an antioxidant by interrupting the chain reaction of lipid peroxidation.
- Vitamin E donates a hydrogen atom to a lipid peroxyl radical, preventing the formation of further radicals.
- Vitamin C acts as an antioxidant by reducing the oxidized form of Vitamin E, allowing it to continue its role as an antioxidant.
Build-Up Of Ammonium Ions In The Brain
- The build-up of ammonium ions in the brain results in ammonia being converted to glutamate.
- This process depletes the brain of α-ketoglutarate.
- α-ketoglutarate is a crucial component of the citric acid cycle, leading to impaired cellular respiration.
- Deficiency of α-ketoglutarate can lead to neuronal dysfunction and cell death.
###Â Urea Cycle Enzyme Deficiencies
- Infant A exhibits signs of a deficiency in the enzyme argininosuccinate lyase, which catalyzes the conversion of argininosuccinate to arginine and fumarate.
- This deficiency can lead to a build-up of argininosuccinate and citrulline.
- Infant B exhibits signs of a deficiency in the enzyme ornithine transcarbamylase, which catalyzes the conversion of ornithine and carbamoyl phosphate to citrulline.
- This deficiency can lead to a build-up of ammonia and glutamine.
Metabolic Pathways
- Pathway A is the Glycolysis.
- Pathway B is the Krebs cycle.
- Pathway C is the Oxidative phosphorylation.
Effect of Diabetes on Metabolic Pathways
- Type 1 diabetes is characterized by the absence of insulin production, which is needed for glucose to enter cells.
- In type 1 diabetes, Pathway B will be non-functional, as glucose cannot enter the Krebs cycle.
- Pathway C also will be non-functional because the Krebs cycle is not producing electron carriers (NADH and FADH2) needed for oxidative phosphorylation.
- However, in the absence of glucose, the body will use alternative sources of energy, including fatty acids and ketone bodies.
Ketone Bodies in Starvation
- The brain can use ketone bodies as an energy source under starvation conditions.
- This is because the brain has a limited supply of glucose and can use ketone bodies when glucose is low.
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Description
Explore the implications of McArdle disease, a condition related to glycogen metabolism. Understand how this deficiency affects muscle energy production, lactate levels, and causes muscle pain and fatigue. This quiz will help reinforce your knowledge of muscle physiology and pathology.
