Endocrinology Quiz: Diabetes and Hormones
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Questions and Answers

A patient with type 1 diabetes mellitus takes an insulin injection before eating dinner but then gets distracted and does not eat. Approximately 3 hours later, the patient becomes shaky, sweaty, and confused. These symptoms have occurred because of which one of the following?

  • Elevated ketone-body levels
  • Increased glucagon release from the pancreas
  • Decreased glucagon release from the pancreas
  • Low blood glucose levels (correct)
  • High blood glucose levels

Concerning our patient in question 19.1, if the patient had fallen asleep before recognizing the symptoms, the patient could lose consciousness while sleeping. If that were to occur and paramedics were called to help the patient, the administration of which one of the following would help to reverse this effect?

  • Normal saline
  • Epinephrine (correct)
  • Short-chain fatty acids
  • Triglycerides
  • Insulin

Caffeine is a potent inhibitor of the enzyme cAMP phosphodiesterase. Which one of the following consequences would you expect to occur in the liver after drinking two cups of strong espresso coffee?

  • A reduced rate of glucose export to the circulation
  • An enhancement of glycolytic activity
  • An inhibition of PΚΑ
  • A prolonged response to insulin
  • A prolonged response to glucagon (correct)

Assume that an increase in blood glucose concentration from 5 to 10 mM would result in insulin release by the pancreas. A mutation in pancreatic glucokinase can lead to MODY because of which one of the following within the pancreatic β-cell?

<p>A reduced ability to raise ATP levels (D)</p> Signup and view all the answers

Which one of the following organs has the highest demand for glucose as a fuel?

<p>Brain (C)</p> Signup and view all the answers

Flashcards

What is the main product of liver glycogen breakdown?

The breakdown of liver glycogen yields glucose and glucose 1-phosphate, with glucose being the primary product.

What enzyme's deficiency causes shorter-than-normal glycogen branches?

A deficiency in amylo-1,6-glucosidase results in the inability to break down α-1,6 glycosidic bonds in glycogen, leading to shorter-than-normal glycogen branches.

What happens to the lactate levels in a patient with a muscle phosphorylase deficiency?

A deficiency in muscle phosphorylase prevents the breakdown of glycogen in muscles, leading to decreased lactate levels during exercise. This is because muscle glycogen cannot be used as a fuel source.

What effect does increased glucose intake have on liver glycogen synthase activity?

Increased glucose intake stimulates glycogen synthase activity in the liver, leading to increased glycogen synthesis.

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What is the activity of glycogen synthase and glycogen phosphorylase in a type 1 diabetic who has not taken insulin for 72 hours?

Under conditions of prolonged insulin deficiency and low glucose intake, the body relies on gluconeogenesis for glucose production. This suppresses glycogen synthesis, and glycogen breakdown is prioritized. Therefore, both glycogen synthase and glycogen phosphorylase are inactive.

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What condition is required for glycogen degradation in muscle with a defective protein kinase A?

Muscle glycogen degradation is primarily stimulated by increased intracellular calcium levels, which activate glycogen phosphorylase. cAMP levels have minimal impact.

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What enzyme is essential for maintaining normal blood glucose levels?

Liver glucose 6-phosphatase is necessary for maintaining normal blood glucose levels by converting glucose 6-phosphate back into glucose for release into the bloodstream.

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Are glycogen synthesis and degradation reversible processes?

Glycogen synthesis and degradation are separate processes that use different enzymes. Synthesis requires UDP-glucose and involves adding glucose units to the non-reducing end of glycogen. Degradation involves breaking down α-1,4 and α-1,6 glycosidic bonds.

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What are some common symptoms of glycogen storage diseases?

Glycogen storage diseases (except type O) do not cause death in infancy or childhood, but they can cause hepatomegaly and hypoglycemia.

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What explains the fluctuations in blood glucose levels in a newborn?

Normal physiological changes at birth include a decrease in blood glucose levels in the first hours due to the baby's adjustment from the maternal glucose supply to its own physiological mechanisms.

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What is glycolysis?

Glycolysis is a metabolic pathway that breaks down glucose to pyruvate, generating ATP and NADH.

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What is the net yield of ATP and NADH from glyceraldehyde 3-P to pyruvate?

The net yield of ATP and NADH from the conversion of glyceraldehyde 3-P to pyruvate is 2 ATP and 2 NADH.

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What is the net yield of ATP and NADH from glucose 1-P to two pyruvate molecules?

The net yield of ATP and NADH from glucose 1-P to two molecules of pyruvate is 3 ATP and 2 NADH.

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How is ATP formed in glycolysis?

ATP is not formed by oxidative phosphorylation in glycolysis. Instead, ATP is generated by substrate-level phosphorylation.

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Which reaction commits glucose to the glycolytic pathway?

The commitment step of glycolysis is the conversion of fructose 6-P to fructose 1,6-bisP, catalyzed by phosphofructokinase-1.

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What intermediate is common in both fructose metabolism and glycolysis?

Fructose 6-P is a common intermediate in both fructose metabolism and glycolysis.

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What is the likely reducing sugar found in the urine of a patient with galactosemia?

The reducing sugar detected in the urine of the 4-week-old baby is likely galactose.

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What can be measured to diagnose galactosemia?

Measuring galactose 1-P levels in the cells can diagnose galactosemia.

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Where is lactate primarily produced?

Lactate is not produced primarily in the liver. Instead, it is generated in tissues with high glycolytic activity, like muscles, and then transported to the liver for gluconeogenesis.

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How do red blood cells generate energy if they lack mitochondria?

Red blood cells rely on glycolysis for ATP production as they lack mitochondria.

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What enzyme's mutation leads to both lactic acidemia and reduced α-ketoglutarate dehydrogenase activity?

A mutation in the E3 subunit of pyruvate dehydrogenase affects the activity of both pyruvate dehydrogenase and α-ketoglutarate dehydrogenase, resulting in impaired TCA cycle activity.

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What metabolic acid accumulates in thiamin deficiency?

Thiamin deficiency leads to the accumulation of pyruvic acid, affecting multiple metabolic pathways, including the TCA cycle.

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Which TCA cycle enzyme is embedded in the inner mitochondrial membrane?

Succinate dehydrogenase is the only TCA cycle enzyme embedded in the inner mitochondrial membrane. This is crucial for electron transport.

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How does exercise affect the TCA cycle activity?

The increased NADH/NAD+ ratio during exercise inhibits TCA cycle activity. This is because NADH is a product of the cycle, and its accumulation signals a slowdown.

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What deficiency would lead to an inability to produce coenzyme A?

Pantothenate is essential for the synthesis of coenzyme A (CoA), which is a key component of the TCA cycle and other metabolic pathways.

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What compound donates eight electrons to the electron transport chain?

Acetyl-CoA contributes eight electrons to the electron transport chain.

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What compound accumulates during hypoxia in cardiomyocytes?

During hypoxia, the TCA cycle slows down, leading to the accumulation of citrate.

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What are the products of acetyl-CoA metabolism in the TCA cycle?

One molecule of acetyl-CoA generates two molecules of CO2, three molecules of NADH, one molecule of FAD(2H), and one molecule of GTP. The GTP is subsequently converted to ATP.

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What are the consequences of a mutation in the α-subunit of E1 in PDC?

An X-linked dominant mutation in the α-subunit of E1 in pyruvate dehydrogenase (PDC) results in a decreased rate of pyruvate oxidation, leading to an increase in plasma lactate and pyruvate concentrations.

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Why does a pyruvate carboxylase deficiency lead to lactic acidemia?

Pyruvate carboxylase deficiency impairs gluconeogenesis, leading to an accumulation of pyruvate that is then converted to lactate by lactate dehydrogenase.

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What component of the electron transport chain is oxidized in the presence of cyanide?

Cyanide inhibits Complex IV (cytochrome oxidase), blocking electron flow and preventing the reduction of oxygen.

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What is the effect of valinomycin on the proton motive force?

Valinomycin allows potassium ions to cross the inner mitochondrial membrane, dissipating the proton gradient and reducing the proton motive force.

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How does dinitrophenol uncouple oxidative phosphorylation?

Dinitrophenol allows protons to leak across the inner mitochondrial membrane, uncoupling electron transport from ATP synthesis.

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Why does iron-deficiency anemia lead to fatigue?

Iron-deficiency anemia impairs the function of the electron transport chain because iron is essential for several complexes, particularly complex I and complex II.

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What is a common characteristic of OXPHOS diseases?

OXPHOS diseases often involve defects in the inner mitochondrial membrane, affecting electron transport and ATP production.

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Which protein complex is minimally affected by reduced heme synthesis?

Lead poisoning can affect heme synthesis, leading to a reduction in heme-containing proteins like hemoglobin and myoglobin. This is because lead interferes with the enzymes involved in heme production.

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What effect does rotenone have on ATP production?

Rotenone inhibits complex I (NADH dehydrogenase), reducing the electron flow through the electron transport chain and leading to decreased ATP production.

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What is a key component of oxidative phosphorylation?

ATP synthase is a key component of oxidative phosphorylation. It utilizes the proton gradient generated by the electron transport chain to synthesize ATP.

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What component of the ETC may be lost due to a high salt solution?

The loss of cytochrome c from the electron transport chain due to disruption of noncovalent interactions in the inner mitochondrial membrane impairs electron transport and leads to a decrease in oxygen consumption.

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What is a potential side effect of activating UCPs?

Activation of UCPs uncouples oxidative phosphorylation, leading to increased heat production and decreased ATP synthesis. This is because UCPs facilitate proton leakage across the inner mitochondrial membrane

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Which of the following vitamins/ enzymes is unable to protect against free radical damage?

Vitamin B6, unlike the other listed vitamins and enzymes, does not directly protect against free radical damage.

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What reaction is catalyzed by superoxide dismutase?

Superoxide dismutase catalyzes the conversion of two superoxide radicals into hydrogen peroxide and oxygen.

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How does vitamin E act as an antioxidant?

Vitamin E acts as an antioxidant by physically interacting with free radicals, preventing them from damaging cellular components. It does this by providing a hydrogen atom to the radical, essentially neutralizing it.

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Which metal promotes the conversion of hydrogen peroxide into dangerous radicals?

In the presence of iron, hydrogen peroxide can be converted into more dangerous radical forms, particularly the hydroxyl radical, through the Fenton reaction.

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Why is mitochondrial DNA more susceptible to oxidative damage?

The mitochondrial membrane is more permeable to ROS than the nuclear membrane, leading to increased oxidative damage to mitochondrial DNA.

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What is the main reactive oxygen species that cannot be generated in patients with chronic granulomatous disease?

Chronic granulomatous disease results from a deficiency in NADPH oxidase, which impairs the ability of phagocytes to produce superoxide, a reactive oxygen species essential for killing pathogens.

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What reactive oxygen species cannot be detoxified in patients with amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis can result from mutations in SOD1, leading to the inability to detoxify superoxide. This deficiency contributes to the accumulation of reactive oxygen species and neuronal damage.

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In high concentrations, what reactive species can NO produce?

Nitric oxide (NO) can lead to the formation of reactive nitrogen--oxygen species (RNOS) at high concentrations. These species can contribute to inflammation and oxidative stress, contributing to several diseases.

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How can xenobiotics increase the risk of free radical injury?

Xenobiotics can induce enzymes containing cytochrome P450. These enzymes can generate reactive oxygen species as a byproduct of their metabolic activity.

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Which food is a good source of antioxidants?

Citrus fruits are a good source of antioxidants, such as vitamin C, which helps protect cells from free radical damage.

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Study Notes

Questions and Answers

  • Question 1: A patient with type 1 diabetes skips dinner after taking insulin. Three hours later, they feel shaky, sweaty, and confused. What caused this?

    • Low blood glucose levels.
  • Question 2: A patient in question 19.1 falls asleep before paramedics arrive. What treatment would help reverse the effect of this?

    • Epinephrine.
  • Question 3: What effect might drinking strong espresso have on the liver?

    • A prolonged response to glucagon.
  • Question 4: A mutation in pancreatic glucokinase can lead to MODY. Which process is affected?

    • A reduced ability to raise ATP levels.
  • Question 5: Which organ has the highest demand for glucose for fuel?

    • The brain.
  • Question 6: Why doesn't glucagon affect muscle metabolism?

    • Muscle cells lack the glucagon receptor.
  • Question 7: A patient experiences tremors, sweating, and a rapid heartbeat. What hormone is likely released?

    • Epinephrine.
  • Question 8: Which meal will have the lowest circulating glucagon shortly after eating?

    • High-carbohydrate meal.
  • Question 9: A 45-year-old patient is in a coma due to severe hyperglycemia. How can you determine if it's type 1 or type 2 diabetes?

    • Measure the C-peptide level.
  • Question 10: High blood glucose levels in a patient's urine lead to cerebral dysfunction. What's the cause?

    • Dehydration.
  • Question 1: Which transporter is most responsible for transporting fructose from the blood into cells?

    • GLUT 5.
  • Question 2: A patient with alcoholism has pancreatitis and discomfort after a high-carbohydrate meal. What is the likely digestive issue?

    • Reduced ability to digest starch.
  • Question 3: A man with type 1 diabetes skips insulin injections. Which tissue will be most affected?

    • Red blood cells.
  • Question 4: What are the main carbohydrate products formed after digesting a cake (flour, milk, sucrose)?

    • Glucose, fructose, and galactose.
  • Question 5: A patient with a genetic defect in intestinal epithelial cells cannot digest disaccharides. What would be elevated?

    • Maltose, sucrose, and lactose in the stool.
  • Question 6: Which carbohydrate contains glucosyl units linked through alpha-1,6 glycosidic bonds?

    • Amylopectin.
  • Question 7: A patient increases fiber intake but experiences abdominal cramping, bloating, and increased flatulence. What's the cause?

    • Bacteria in the colon convert fiber to H2, CO2, and methane.
  • Question 8: A newly diagnosed patient with diabetes avoids table sugar but consumes fruits, fruit drinks, milk, honey, and vegetables, leading to poor diabetic control. What form of carbohydrate is primarily in their diet?

    • Glucose.
  • Question 9: A 10-year-old develops severe diarrhea and now has digestive issues after drinking milk. What's the best advice?

    • Her ability to drink milk should return in a few days.
  • Question 10: A runner wants a high glycemic index food for "carb loading". What should they choose?

    • Malted milk balls.
  • Question 1: What is the body's universal fuel used by virtually all tissues?

    • Glucose.
  • Question 2: What is a major role of glycolysis?

    • To generate energy.
  • Question 3: What is the net yield of ATP and NADH from one glucose 1-P to two pyruvate molecules?

    • 2 ATP, 2 NADH.
  • Question 4: What statement correctly describes an aspect of glycolysis?

    • Pyruvate kinase is the rate-limiting enzyme.
  • Question 5: What is found in both the fructose metabolic pathway and the glycolytic pathway?

    • Glyceraldehyde 3-P.
  • Question 6: A 4-week-old baby has frequent vomiting, tenderness, enlarged liver, and hints of cataracts. A urine dipstick test shows a positive result for a reducing sugar. What is the likely compound?

    • Glucose.
  • Question 7: What intracellular metabolite allows for determination of an enzyme deficiency in the child from question #6?

    • Fructose 6-P.
  • Question 8: What is one action of metformin in treating type 2 diabetes and why does it not cause lactic acidosis in practice?

    • Decreases hepatic gluconeogenesis. The Cori cycle deals with the lactate buildup in the liver.
  • Question 9: What reaction commits glucose to the glycolytic pathway?

    • Glucose to glucose 6-P.
  • Question 10: How do red blood cells generate energy to maintain ion gradients?

    • Substrate-level phosphorylation.
  • Question 1: What enzyme is unique in the TCA cycle?

    • Succinate dehydrogenase
  • Question 2: What is the effect of valinomycin and potassium ions on the proton motive force (PMF)?

    • PMF is reduced to zero.
  • Question 3: How does dinitrophenol (DNP) uncouple oxidative phosphorylation?

    • Blocking proton transport.
  • Question 4: Why might a 25-year old woman have chronic fatigue due to iron-deficiency anemia?

    • Iron's vital role in electron transfer in the electron transport chain is impaired.
  • Question 5: What is a characteristic finding in OXPHOS diseases?

    • High NADH/NAD+ ratio in the mitochondria.
  • Question 6: What protein complex is unaffected by lead poisoning interfering with heme synthesis?

    • Complex IV.
  • Question 7: Rotenone inhibits NADH dehydrogenase. What's the effect on ATP production?

    • A 50% reduction in ATP production.
  • Question 8: Which compound is a crucial component of oxidative phosphorylation?

    • ATP, which is produced through oxidative phosphorylation.
  • Question 9: What component of the electron transport chain is lost when mitochondria are incubated with a high salt solution?

    • Cytochrome C
  • Question 10: What could be a potential side effect of drugs that activate multiple uncoupling proteins (UCPs)?

    • Increased body temperature
  • Question 1: What vitamin or enzyme cannot protect against free-radical damage?

    • Vitamin B6
  • Question 2: What reaction is catalyzed by superoxide dismutase?

    • 2O2- + 2H+ → H2O2 + O2
  • Question 3: How does vitamin E act as an antioxidant?

    • Vitamin E stabilizes free radicals by forming a covalent bond.
  • Question 4: What metal can convert hydrogen peroxide to dangerous forms?

    • Iron.
  • Question 5: Why is oxidative damage to mitochondrial DNA likely greater than nuclear DNA?

    • Mitochondrial membranes are permeable to reactive oxygen species (ROS).
  • Question 6: What is the primary defect in chronic granulomatous disease?

    • Inability to generate superoxide
  • Question 7: What process is impaired in patients with amyotrophic lateral sclerosis (ALS), possibly inherited from a father with ALS?

    • Detoxification of oxidized glutathione
  • Question 8: How do compounds like nitroglycerin work, and what diseases can be affected by highly concentrated nitric oxide?

    • Form Nitric Oxide (NO), a vasodilator. They form reactive nitrogen-oxygen species (RNOS) which are involved in ischemic heart disease and infertility.
  • Question 9: How can foreign compounds like alcohol increase the risk of free-radical injury?

    • Induction of enzymes like cytochrome P450.
  • Question 10: Which food is high in antioxidants?

    • Citrus fruits.
  • Question 1: What does liver glycogen degradation produce during glucagon release?

    • More glucose 1-phosphate than glucose.
  • Question 2: What protein activity is impaired in a patient with abnormal glycogen branching patterns?

    • Glycogenin.
  • Question 3: How does a deficiency in muscle phosphorylase affect exercising patients?

    • Patients exhibit decreased lactate levels in blood drawn from the forearm.
  • Question 4: What process is likely enhanced in a normal glucose tolerance test?

    • Glycogen synthase activity.
  • Question 5 (F): Why does glycogen degradation occur under high intracellular calcium levels when muscle protein kinase A is affected by cAMP?

    • Calcium is a secondary messenger independent of cAMP.
  • Question 6: What is crucial for maintaining blood glucose levels?

    • Liver glucose-6-phosphatase

-Question 7: What is the correct statement about glycogen synthesis and degradation?

  • Glycogen uses the same enzymes in both processes, meaning some steps are reversible.

  • Question 8 (mutations): What is true about glycogen storage diseases (except type O)?

    • Type O is the only one that doesn't primarily affect the liver.
  • Question 10 (newborn): What is most likely given to a newborn with glucose levels of 50 mg/dL in the first hour and 80 mg/dL two hours after delivery?

    • IV dextrose 50 to increase blood glucose.

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Test your knowledge on endocrinology with this quiz focusing on diabetes, hormone functions, and metabolic processes. Questions cover various scenarios involving insulin, glucagon, and their effects on the body. Perfect for medical students and health professionals.

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