Glycogen Metabolism
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A researcher is investigating metabolic pathways and aims to inhibit a specific step using a drug. According to the information presented, which aspect of metabolic pathways would be most relevant for the researcher to consider when selecting a drug?

  • The specific enzymes that act as regulatory steps. (correct)
  • The final products of the metabolic pathway.
  • The effect of the drug on tissues within the liver.
  • The stimulators that work by hormones to increase blood sugar.

Imagine a cell is exposed to a toxin that disrupts the function of mitochondria. Which metabolic processes would likely be most immediately affected?

  • Glycolysis and the pentose phosphate pathway.
  • Glycolysis and fatty acid synthesis.
  • PP pathway and fatty degradation.
  • TCA cycle and fatty acid oxidation. (correct)

Consider a patient showing clinical signs associated with an enzyme deficiency that affects the conversion of a substrate into a product. What broader clinical effects should be analyzed?

  • The downstream effects of the deficient enzyme.
  • The accumulation of the substrate and use of alternative pathways. (correct)
  • There is increased alternate pathway and the product is being synthesized.
  • The vitamin level affects conversion of the deficient enzyme.

A patient is diagnosed with a genetic defect affecting an enzyme in a metabolic pathway. Which downstream effect should be analyzed when determining the clinical implications?

<p>The accumulation of the substrate for that enzyme. (B)</p> Signup and view all the answers

A researcher discovers a new drug that enhances the activity of GLUT4 transporters in cells. For which condition would this drug be most applicable, considering the primary function of GLUT4?

<p>Primarily glucose-4 in adipose tissue and muscle. (A)</p> Signup and view all the answers

In order to study the liver's response to elevated blood glucose and understanding its role as a 'glucose sensor,' which enzyme should be considered?

<p>Glucokinase (high Km). (A)</p> Signup and view all the answers

If a researcher is examining the metabolic changes in a patient's liver during prolonged fasting. Which alterations in active pathways would the researcher expect to observe?

<p>Activation of glucagon and ribulose-5. (A)</p> Signup and view all the answers

A researcher inhibiting the pentose phosphate pathway to examine the effects on nucleotide synthesis. Which metabolic intermediate is required?

<p>Ribose 5-phosphate. (C)</p> Signup and view all the answers

If a patient has hemolytic anemia due to a G6PD deficiency. Which product would be affected?

<p>Reduced glutathione. (B)</p> Signup and view all the answers

If a researcher is investigation of a drug company, a key reaction to administer will be Vitamin B1?

<p>A key reaction to increase transketolase. (C)</p> Signup and view all the answers

A scientist is engineering a yeast strain for alcoholic fermentation. Which enzyme should be cytosolic?

<p>Glocolysis. (D)</p> Signup and view all the answers

Which process has a primary role during the energy generation phase of glycolysis?

<p>Glyceraldehyde 3-phosphate. (B)</p> Signup and view all the answers

A drug inhibits phosphoglycerate kinase. How does this affect the overall process of glycolysis?

<p>Substrate level phosphorylation will decrease. (B)</p> Signup and view all the answers

A biological research team is studying the metabolic adaptations in a marine mammal that spends extended periods under water. How is the generation of lactate important?

<p>Used in the absence of oxygen when needing NAD+. (B)</p> Signup and view all the answers

In a study on muscle metabolism during intense exercise, a researcher measures a significant increase in blood lactate levels. Which processes occur in the presence of oxygen abundant of glucose?

<p>Produces glucose to 2 pyruvate. (B)</p> Signup and view all the answers

Consider muscle cells during strenuous activity, resulting in muscle fatigue. What metabolic adaptation decreases cramping?

<p>Decreased blood lactate in blood. (C)</p> Signup and view all the answers

A study shows aerobic and anaerobic exercises are measured. Which result correlates with ATP producing the fastest vs. highest ammount?

<p>Aerobic exercise: Maximal ATP and abundant mitchondria. Anaerobic exersize: Cytosol Glycolysis with minimal ATP. (A)</p> Signup and view all the answers

During a physiology experiment, muscle tissue is studied during both aerobic and anaerobic conditions. How is the metabolism different?

<p>The accumlation of lactic acid will allow the conversion of pyruvates. (A)</p> Signup and view all the answers

A marathon runner relies on the Cori cycle to maintain blood sugar. What process is expected to occur in the muscles and the liver?

<p>Muscles produce lactate that liver converts to glucose. (D)</p> Signup and view all the answers

A researcher studies of Tarui disease. What would be expected to be a result in the absence of anaerobic testing?

<p>Elevated glycolytic muscles following high deficiency. (A)</p> Signup and view all the answers

Arsenic poisoning affects glyceraldehyde 3-phosphate dehydrogenase. As a result, which process is affected?

<p>The mitochondrial enzymes are effected. (A)</p> Signup and view all the answers

If a patient is unable to convert pyruvates into lactate, which area in cells will be effected?

<p>It effects cytoplasm and cytosol Lactate. (D)</p> Signup and view all the answers

Analysis of a patient with lactic acidosis reveals an elevated presence of lactate. Which set of conditions contribute to the condiction in relation to NADH?

<p>Increased NADH/NAD with Binge alcohol intake with deficieny of cori. (C)</p> Signup and view all the answers

For a researcher examining a diabetic neuropathic effects, which would be a key action to limit energy ATP source and tumor cells?

<p>Decrease synthesis of triacylglycerol and wound healing. (C)</p> Signup and view all the answers

A pharmaceutical company is developing drugs that target specific locations within cells to treat various metabolic disorders. Considering the information provided, which cellular location would be the most relevant target for a drug designed to enhance glycolysis?

<p>The cytosol, where the enzymes of glycolysis are located. (D)</p> Signup and view all the answers

In a research setting, scientists aim to manipulate metabolic fluxes to enhance the production of a certain metabolite using drugs. Considering the role of regulatory steps in metabolic pathways, how would regulatory steps assist?

<p>Regulatory steps influence the direction and speed of metabolic traffic. (D)</p> Signup and view all the answers

A research team is investigating a newly identified genetic disorder that affects an enzyme responsible for catalyzing a reaction with a water-soluble vitamin as a coenzyme. What approach should be undertaken to understand the effects?

<p>Identify which water-soluble vitamins are required. (D)</p> Signup and view all the answers

A graduate student is creating a model to simulate the metabolic response of the liver under different hormonal conditions, specifically changes in glucose metabolism. Which consideration of hormonal modulation is most accurate?

<p>The balance between insulin and glucagon impacting liver pathways. (D)</p> Signup and view all the answers

A researcher is studying the effects of a specific drug on tissues with varying metabolic activities, notably focusing on the differential impact on ATP production. Which factor would help most, considering cytosol?

<p>The occurrence of glycolysis. (C)</p> Signup and view all the answers

When studying a disease related to the deficiency in the role of glucose 6-phosphate dehydrogenase, that results in lower amounts of NADPH. What is the best result to measure?

<p>Increase in superoxides. (D)</p> Signup and view all the answers

A patient shows low ATP production, and hemolytic anemia caused by low NADPH. In which area is the gene affected?

<p>Fatty-acid biosynthesis. (A)</p> Signup and view all the answers

A patient has a low red blood cell count, caused by reactive oxygen intermediates. What action will occur?

<p>Glutathione in red cells will be effected. (A)</p> Signup and view all the answers

A geneticist is tracing the heritability of metabolic defects within a large family, noting a pattern of disease that appears in nearly every generation, but only through the maternal line. Which metabolic disease aligns with inheritance?

<p>Mitochondrial disorder. (B)</p> Signup and view all the answers

A community health initiative focuses on the importance of TPP from Vitamin B for those developing countries. What area is TPP vitamin B1 important for??

<p>Transketolase activity (D)</p> Signup and view all the answers

A biology student is learning about various metabolic pathways, specifically how different sugars are metabolized and interconnected. How is glucose connected vs galactose?

<p>Galactose produces glucose 1-P. (D)</p> Signup and view all the answers

A biochemical engineering student isolates a novel bacterial strain to be used for industrial production of ethanol. Which area is key for production?

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

A researcher is investigating how cancer cells adjust their metabolism, specifically focusing on ATP generation pathways when oxygen is limited. When a tumor inhibits mitochondria, what process still generates energy??

<p>Glycolysis in the cytosol. (D)</p> Signup and view all the answers

During a study on metabolic disorders affecting energy production in muscle cells, a researcher observes that a particular enzyme deficiency leads to muscle fatigue and cramping during strenuous exercise. What would result in the muscles?

<p>A decrease in the glycolytic rate. (B)</p> Signup and view all the answers

Aerobic and anaerobic conditions that is oxygen has the largest impact vs. fastest ATP

<p>Aerobic makes highest ammount slower, and anerobic makes less and faster. (B)</p> Signup and view all the answers

During a biochemical experiment, muscle tissue is analyzed under both aerobic and anaerobic conditions to assess metabolic differences. How is the metabolism expected to vary?

<p>In aerobic you can use both pyruvate and oxygen abundant vs anerobic you convert to lactate. (D)</p> Signup and view all the answers

A long-distance runner relies on the Cori cycle during a marathon. This cycle involves metabolic interactions between the muscles and liver. Which reactions need to occur?

<p>Muscle produces lactate transported for processing into liver. (C)</p> Signup and view all the answers

Based on the information given about Tarui disease and its effect anaerobic results vs. aerobic, what result occurs?

<p>NO increase in blood Lactase + Cramps. (C)</p> Signup and view all the answers

A biochemist investigates the effects of arsenic poisoning, focusing on its impact on enzymes within key metabolic pathways. How does the effect of Arsenic affect the metabolic system?

<p>Arsenic inhibits complex enzyme and ATP production. (B)</p> Signup and view all the answers

If someone is unable to convert pyruvate into other products such as OAA, Alanine, Lactate or Acetyl-CoA, which location in cells will be affected?

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

Analysis of a patient with lactic acidosis has an increased amount of lactic acid. Which process contribute to the condition in the absence of oxygen?

<p>Increased concentration of NADH and lactate production. (D)</p> Signup and view all the answers

From the fate of absorbed glucose: Which is the target for facilitated diffusion of glucose?

<p>GLUT2 Transporter. (D)</p> Signup and view all the answers

A molecular biologist is researching factors influencing the expression of genes involved in cellular respiration, especially in conditions of fluctuating glucose availability. What best explains high km role??

<p>Glucokinase, liver and pancreas cells. (D)</p> Signup and view all the answers

A researcher studies fates of glucose 6 phosphate in the human liver. Which product is the result??

<p>Glycogenolysis creates the products. (A)</p> Signup and view all the answers

Imagine a scientist is trying to design a new drug that specifically targets the metabolic pathways in liver cells to reduce fatty acid synthesis. What would be the focus of reduction?

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

If in a diagnosis, there is mutations of NADPH oxidase it leads to?

<p>Chronic granulomatous disease. (C)</p> Signup and view all the answers

A research study is looking for an inhibitor for superoxides. What do they need to examine?

<p>NADPH Oxidase. (D)</p> Signup and view all the answers

A graduate student is engineering glycolic enzymes. How can he affect the amount of products from the liver and brain?

<p>Change the rate. (D)</p> Signup and view all the answers

Suppose skeletal muscles are contracting. What has to happen to use glucose for energy?

<p>Needs to be from Glycogen or Glucose. (D)</p> Signup and view all the answers

During strenuous activity, blood lactate levels increased because of increased glucose to create ____ ATP.

<ol start="2"> <li>(B)</li> </ol> Signup and view all the answers

There is a patient with PFK deficiency. What would NOT increase?

<p>Blood Lactate Levels. (A)</p> Signup and view all the answers

Arsenic binds glyceraldehyde-3-phosphate and affects the metabolic pathway. What is the effect?

<p>Glycolysis would be effected and reduce NADH production. (D)</p> Signup and view all the answers

A doctor suspects their patient with lactic acidosis may have low blood supply. Which option creates an issue from low blood supply?

<p>Embolism. (D)</p> Signup and view all the answers

A scientist is studying the effects of epinephrine on various types of muscle tissue, including cardiac muscle. What would affect or relate?

<p>Wound Healing. (D)</p> Signup and view all the answers

From development from the gut tube: What does the midgut consist of?

<p>Proximal small intestine. (C)</p> Signup and view all the answers

During embryogenesis, a defect during stomach development leads to incomplete rotation along its longitudinal axis. Which clinical finding would you expect in this instance, given the impact on anatomical positioning?

<p>Anterior will become posterior. (D)</p> Signup and view all the answers

In the development of the stomach and pyloric stenosis to what is what direction the axis going??

<p>Top left - Bottom right. (B)</p> Signup and view all the answers

During a prenatal ultrasound, a doctor finds that a baby has nonbilious contents, this means:

<p>There is still bile secretion but there is a problem before stomach. (D)</p> Signup and view all the answers

What issue occurs with the liver?

<p>Gut is suspended. (D)</p> Signup and view all the answers

During an autopsy, it is found that it's an unusual attachment. Which messentary is not working??

<p>Ventral can't attach. (A)</p> Signup and view all the answers

In early fetal development, if you find defects in duodenum, what would occur

<p>There is an issue and requires immediate solution. (A)</p> Signup and view all the answers

Stenosis (due to partial failure of (re) canalization after epithelial proliferation in lumen) what area will be affected in order?

<p>starts but fails to keep going. (B)</p> Signup and view all the answers

When looking at the defects in the duodenum what will happen if there is vomit with bile stain??

<p>An incomplete recanalization in 3rd/4th part. (D)</p> Signup and view all the answers

What is not related to duodenal atresia?

<p>Low pH. (D)</p> Signup and view all the answers

A 5-year-old presents with malabsorption, a noticeable abdominal mass, and imaging reveals a twisted section of the small intestine connected to the ileum. A long term can manifest?

<p>Intestine can be blocked. (D)</p> Signup and view all the answers

A baby is born with development in 6 th week that caused the umbilical cord to be outside of the body. Is this issue:

<p>Is an Umbilical Hernia. (C)</p> Signup and view all the answers

If Binds together?

<p>bifid ventral pancreatic bud. (D)</p> Signup and view all the answers

What is the result in the abnormalities from midgut (in the 10th week)?

<p>Central and left part of abdomen. (A)</p> Signup and view all the answers

With a patient coming back after a few weeks. What action would have occured after and cause the stomach to be the way its

<p>Reappears Lumen (By End embryo period). (B)</p> Signup and view all the answers

When looking at duodenal atrisia, which is not considered?

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

During a physical exam, what result would you expect on the patient?

<p>Encircle Duogenium. (C)</p> Signup and view all the answers

How would you describe a spleen from a slide?

<p>Spleen is mesenchyme. (B)</p> Signup and view all the answers

During prolonged fasting, the body shifts its metabolic focus to maintain blood glucose levels. Which of the following metabolic processes is upregulated in the liver to achieve this?

<p>Gluconeogenesis, synthesizing glucose from non-carbohydrate sources. (C)</p> Signup and view all the answers

Consider a patient with a deficiency in glucose 6-phosphate dehydrogenase (G6PD). This deficiency directly impairs the:

<p>production of NADPH in the pentose phosphate pathway. (C)</p> Signup and view all the answers

A researcher is investigating new drugs that can modulate metabolic pathways. Targeting regulatory enzymes of glycolysis. They can control the:

<p>direction of metabolic flux through the pathway. (B)</p> Signup and view all the answers

Imagine a patient presents with symptoms of muscle weakness, fatigue, and cramping during exercise, but their blood lactate levels do not rise appropriately post-exercise. Which enzyme is affected?

<p>Muscle glycogen phosphorylase. (D)</p> Signup and view all the answers

During intense exercise, muscle cells primarily rely on anaerobic glycolysis for ATP production. This process leads to the formation of:

<p>Lactate. (A)</p> Signup and view all the answers

A patient has no GLUT 4. Due to this, the patient might develop:

<p>Reduced ability of muscle and adipose tissue to take up glucose. (D)</p> Signup and view all the answers

In liver cells, which enzyme is activated when the concentrations of blood glucose are elevated?

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

Which of the following statements accurately describes the metabolic role of the pentose phosphate pathway (PPP)?

<p>Produces ribose-5-phosphate. (D)</p> Signup and view all the answers

A person taking blood samples shows glucose readings even while not eating. What is primarily used to contribute to their blood glucose?

<p>Amino Acids. (C)</p> Signup and view all the answers

In the development of the stomach, rotation along the longitudinal axis is key to its final positioning. What change is responsible?

<p>The right surface becomes posterior. (C)</p> Signup and view all the answers

During the development of the duodenum, various malformations can occur due to incomplete recanalization. What type of vomit suggests if there is an incomplete formated part?

<p>Usually bile stained. (D)</p> Signup and view all the answers

A patient with gastric discomfort finds his stomach axis isn't running above left to below right. Which development of rotation is affected?

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

In a developing fetus, the midgut undergoes a series of processes that include elongation, herniation, and rotation. During which week of gestation does physiological herniation of midgut occur?

<p>6th week. (A)</p> Signup and view all the answers

A newborn presents with nonbilious projectile vomiting. Diagnostic imaging reveals a significant thickening of the pylorus. The axis is?

<p>Short Axis (B)</p> Signup and view all the answers

Several weeks after birth, an examination discovers the baby with a long term twisted section of the small intestine connected to the ileum. Which issue results?

<p>The twisting of the bowel may cause malabsorption. (A)</p> Signup and view all the answers

What is the most common origin in tumors?

<p>Epithelial origin. (A)</p> Signup and view all the answers

Spleen originates from mesenchymal cells of what?

<p>dorsal mesogastrium. (A)</p> Signup and view all the answers

The primary function of the hepatic portal vein is:

<p>transport venous blood from digestive. (A)</p> Signup and view all the answers

In the rare case of there being a persistent remnant of the omphaloenteric duct what does it cause?

<p>A fibrous tube that connects tissue to the organ. (D)</p> Signup and view all the answers

If a structure causes the other to bind to its portion, it will then result in

<p>Atrisia or Ring. (D)</p> Signup and view all the answers

What is the result of issues with the vagus nerve and rotation?

<p>Stays to what wall it stays on. (B)</p> Signup and view all the answers

What is most important to remember when it comes to blood supply to anal canal with its portions, embryonic origin?

<p>It is important for its lymphatic and nervous system. (D)</p> Signup and view all the answers

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Study Notes

Basic Principles of Medicine 2 - Digestion and Metabolism: Glycogen Metabolism

  • Glycolysis and pentose phosphate pathway and their role in metabolism.

Intermediary Metabolism

  • Metabolic map showing the overview of metabolism in the fed state and during fasting.
  • SOM. MK.I. BPM2.3. DM.2. BCHÎœ.1089
  • Lippincott's figure 8.2

Metabolic Pathways

  • Effect of hormones like insulin and glucagon during fed and fasting states.
  • Tissues in which pathway is active and location in the cell.

Inherited Enzyme Deficiencies

  • Deficiency of the deficient enzyme that affects the substrate, coenzyme, or product

Overview of Absorption and Transport

  • Glucose transport in cells is facilitated by GLUT.
    • GLUT 2 present in the liver
    • GLUT 1 and 3 present in neurons and brain
    • GLUT 1 present in erythrocytes
    • GLUT 4 present in adipose tissue and muscle, and is insulin-responsive

Formation of Glucose-6-Phosphate

  • Glucokinase is present in the liver and pancreatic B-cells (high Km)
    • More active when blood glucose elevates; 'Glucose sensor'
  • Other tissues, like hexokinase, have low Km.
  • Glucokinase mutations may cause hyperglycemia and monogenic diabetes / MODY-2
    • B-cells of pancreas cannot respond to high glucose levels.

Fates of Glucose-6-Phosphate in Liver:

  • Activated pathways are different depending on if Insulin vs Glucagon is the primary factor.
  • During insulin-high / fed state:
    • Gluconeogenesis is inhibited.
    • Glycogenolysis is inhibited.
    • Glycolysis is activated.
    • Glycogenesis is activated.
  • During Glucagon-high / fasting, the reverse is true.
  • SOM. MK.I. BPM2.3. DM.2.BCHM.1089

Metabolic Characteristics of the Pentose Phosphate Pathway and NADPH:

  • Cytosol
  • Tissues actively synthesizing:
    • The liver
    • Adipose tissue
    • Adrenal cortex
    • Gonads
  • Erythrocytes
  • Fatty-acid and steroid syntheses
  • Drug metabolism including:
    • Glutathione reduction
    • Generation of superoxide in phagocytes by NADPH oxidase

Role of Glucose-6-Phosphate Dehydrogenase:

  • The rate-limiting step.
  • Glucose 6-phosphate dehydrogenase (G6PD) deficiency
    • The creation of NADPH is inhibited, leading to reactive oxygen intermediates and hemolytic anemia

Pentose Phosphate Pathway Points to Review:

  • Glucose-6-phosphate dehydrogenase forms NADPH.
  • Fatty acid biosynthesis, cholesterol biosynthesis, and steroid hormone synthesis are active
  • Forms ribose phosphate for purine and pyrimidine synthesis
  • Transketolase requires TPP (thiamine)
    • Thiamine deficiency leads to low transketolase activity

Glycolysis points to review:

  • Generates ATP (energy)
  • In the brain (fed and fasted), Actively contracting skeletal muscle.
  • Requires cytosol.
  • Glycolytically enzymes are present in cytosol.

###Aerobic/Anaerobic

  • Different processes take place under different conditions

Overview of phases of glycolysis

  • In stage 1; the body invests Energy in the form of 2 ATP.
  • In stage 2; the Cleavage of 6C sugar into 3C occur to form intermediates.
  • In stage 3; Energy in the form of ATP and NADH is generated.
  • SOM. ΜΚ.Ι. BPM2.3. DM.2.BCHÎœ.1089

Stages involved in Glycolysis

  • Aerobic glycolysis
  • Anaerobic glycolysis

ATP Formation Information:

  • ATP is formed directly from ADP
  • An ETC or Oxygen atmosphere is NOT required
  • Only reactions that for ATP cells that lack mitochondria/hypoxia utilize
  • High energy compounds
    • 1,3-bisphosphoglycerate
    • Phosphoenolpyruvate

Fates of Pyruvate, and relevant enzyme:

  • Aerobic uses Oxygen and is coupled to mitochondria
    • Acetyl CoA
    • pyruvate dehydrogenase complex
  • Anaerobic uses no oxygen & no Mitochondria
    • Lactate: Lactate dehydrogenase
  • This process is impaired without correct mitochondrial counts and correct levels of pyruvate dehydrogenase enzyme

Anaerobic Fate of Pyruvate Information:

  • NADH will form, via glyceraldehyde is reoxidized to form NAD+
  • LDH is cytosolic enzyme Direction of reaction: NADH/NAD+ ratio
  • Identified in muscle (fast-twitch) and liver High NADH increases lactate

Energetics of Aerobic/Anaerobic

  • Aerobic reaction:

    • GlucosePyruvate -= (glyceraldehyde ATP –Refer Shuttles and OxPhos (substrate level phosphorylation) ATP ATP
  • Anaerobic reaction:

    • 3:3 → Lactate used in lactate dehydrogenase 2 from a BPM2.3 dm 2.BCHM.1084 ==End of Text transcript==

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Overview of Glycogen Metabolism, including Glycolysis, the pentose phosphate pathway, and metabolic maps. Discusses the effect of hormones like insulin and glucagon during different states. Covers inherited enzyme deficiencies and glucose transport in cells.

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