Questions and Answers
What specific enzyme deficiency is responsible for McArdle's disease and what symptoms does it cause during exercise?
McArdle's disease is caused by a deficiency in muscle glycogen phosphorylase, leading to muscle cramps and weakness during exercise.
Explain why muscle cells cannot release glucose into the bloodstream.
Muscle cells lack the enzyme glucose-6-phosphatase, preventing them from converting glucose-6-phosphate to free glucose for release into the bloodstream.
What is the main purpose of glycogenolysis in the human body?
The main purpose of glycogenolysis is to break down glycogen into glucose or glucose-6-phosphate to meet the body's energy needs and maintain blood glucose levels.
In which cellular location does the pentose phosphate pathway occur, and what are its two primary functions?
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Describe the role of hormones in the regulation of glycogenolysis.
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What are the symptoms of Hers disease and what enzyme is deficient in this condition?
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Identify the key enzymes involved in glycogenolysis and their specific functions.
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How does the pentose phosphate pathway contribute to maintaining redox balance in the cell?
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What role does NADPH play in red blood cells, and why is its production significant?
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Explain how a cell adjusts its metabolic priorities when there is a high demand for ribose-5-phosphate.
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Describe the impact of glucose-6-phosphate dehydrogenase deficiency on oxidative stress in affected individuals.
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Identify the key phases of the pentose phosphate pathway and their primary functions.
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How does the pentose phosphate pathway contribute to cancer cell metabolism?
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Explain the regulatory mechanism of the pentose phosphate pathway, focusing on the NADP⁺/NADPH ratio.
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What is the significance of the non-oxidative phase in terms of cellular flexibility?
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List the key enzymes involved in the pentose phosphate pathway and their importance.
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What is the primary purpose of gluconeogenesis?
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Identify and explain one precursor for gluconeogenesis.
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How does gluconeogenesis differ from glycolysis in terms of energy requirement?
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Which two enzymes are primarily involved in the conversion of pyruvate to phosphoenolpyruvate (PEP)?
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Describe the role of glycerol in gluconeogenesis.
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Why is it important that glycolysis and gluconeogenesis are reciprocally regulated?
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What is the significance of glucogenic amino acids in gluconeogenesis?
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In which cellular compartments do the majority of gluconeogenesis steps occur?
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What is the primary hormone responsible for stimulating glycogenesis and how does it affect glycogen synthase?
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Explain the role of glucose-6-phosphate in the regulation of glycogenesis.
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How does glycogenesis in muscle tissue differ from that in liver tissue in terms of glucose availability?
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What is the significance of glycogenesis for maintaining blood glucose levels during fasting?
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Describe how ATP functions as an allosteric regulator in glycogenesis.
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What are glycogen storage diseases and what can result from defects in glycogen metabolism enzymes?
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Identify the key enzymes involved in glycogenesis and their respective roles.
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What ensures the rapid mobilization of stored glucose in the liver during periods of low glucose availability?
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What are the two main phases of glycolysis, and what occurs in each phase?
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Explain the significance of phosphofructokinase-1 (PFK-1) in glycolysis.
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Describe the role of ATP in the Energy Investment Phase of glycolysis.
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What are the end products of glycolysis, and how many molecules of each are produced from one glucose molecule?
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Identify the enzyme responsible for the isomerization of glucose-6-phosphate and its role in glycolysis.
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What happens during the cleavage step of fructose-1,6-bisphosphate, and which enzyme facilitates this process?
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In glycolysis, what occurs during the oxidation of G3P, and which enzyme is involved?
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How does the conversion of DHAP into G3P affect the overall yield of G3P from one glucose molecule?
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Study Notes
Overview of Glycolysis
- Glycolysis converts one molecule of glucose (six carbons) into two pyruvate molecules (three carbons each).
- Produces ATP and NADH, which are utilized in subsequent metabolic pathways.
Phases of Glycolysis
- Energy Investment Phase: Consumes ATP to modify glucose for later steps.
- Energy Payoff Phase: Produces ATP and NADH by converting intermediates to pyruvate.
Energy Investment Phase (Steps 1-5)
-
Glucose Phosphorylation:
- Enzyme: Hexokinase; ATP phosphorylates glucose to form glucose-6-phosphate (G6P).
- Traps glucose inside the cell.
-
Isomerization:
- Enzyme: Phosphoglucose isomerase; converts G6P (six-membered) to fructose-6-phosphate (F6P) (five-membered).
-
Second Phosphorylation:
- Enzyme: Phosphofructokinase-1 (PFK-1); F6P is phosphorylated to fructose-1,6-bisphosphate (F1,6BP) using ATP.
- Key regulatory step in glycolysis.
-
Cleavage of Fructose-1,6-bisphosphate:
- Enzyme: Aldolase; splits F1,6BP into glyceraldehyde-3-phosphate (G3P) and dihydroxyacetone phosphate (DHAP).
-
Isomerization of DHAP:
- Enzyme: Triose phosphate isomerase; converts DHAP into G3P, generating two G3P molecules from one glucose.
Energy Payoff Phase (Steps 6-10)
-
Oxidation of G3P:
- Enzyme: Glyceraldehyde-3-phosphate dehydrogenase; oxidizes G3P producing NADH and 1,3-bisphosphoglycerate (1,3BPG).
Overview of Gluconeogenesis
- Main purpose: Maintain blood glucose levels for glucose-dependent tissues (e.g., brain, red blood cells).
- Precursors include lactate, glycerol, and amino acids (especially alanine and glutamine).
Key Differences Between Glycolysis and Gluconeogenesis
- Glycolysis: Breaks down glucose to produce ATP.
- Gluconeogenesis: Synthesizes glucose, typically requiring ATP.
- Regulated reciprocally to prevent futile cycles.
Precursors for Gluconeogenesis
- Lactate: Converted back to pyruvate in the liver via the Cori cycle.
- Amino Acids: Glucogenic amino acids converted to intermediates; alanine to pyruvate.
- Glycerol: From triglyceride breakdown, entering gluconeogenesis as dihydroxyacetone phosphate (DHAP).
Bypassing the Irreversible Steps of Glycolysis
- Conversion of pyruvate to phosphoenolpyruvate (PEP) involves:
- Pyruvate carboxylation to oxaloacetate (OAA) by Pyruvate carboxylase in mitochondria, requiring ATP and CO₂.
Regulation of Glycogenesis
- Regulated by hormonal signals (insulin) and allosteric mechanisms.
- Insulin stimulates glycogenesis by activating glycogen synthase and inhibiting glycogen phosphorylase.
Role of Glycogenesis in Different Tissues
- Liver: Stores glycogen to maintain blood glucose levels between meals; can convert glycogen back to glucose.
- Muscle: Uses glycogen locally for energy; glycogen is not released into the bloodstream.
Importance of Glycogenesis
- Energy storage for excess glucose.
- Regulates blood glucose and provides energy during physical activity.
Glycogen Storage Diseases (GSD)
- Genetic defects lead to abnormal glycogen metabolism; symptoms include muscle weakness and hypoglycemia.
- Examples: McArdle's disease (muscle glycogen phosphorylase deficiency) and Hers disease (liver glycogen phosphorylase deficiency).
Summary of Glycogenesis
- Purpose: Store glucose as glycogen when levels are high.
- Location: Primarily in liver and muscle.
- Key Enzymes: Glycogen synthase and branching enzyme.
Glycogenolysis Summary
- Purpose: Break down glycogen to glucose/glucose-6-phosphate for energy needs.
- Location: Liver and muscle.
- Key Enzymes: Glycogen phosphorylase and debranching enzyme.
Importance of Glycogenolysis
- Mobilizes stored glucose to maintain blood glucose levels during fasting or provide energy during exercise.
Pentose Phosphate Pathway (PPP)
- A metabolic pathway parallel to glycolysis.
- Functions: Produces NADPH for biosynthetic reactions and ribose-5-phosphate for nucleotide synthesis.
Overview of the Pentose Phosphate Pathway
- Occurs in the cytoplasm, inhibited by high NADPH levels.
- Flexible in responding to cellular needs for NADPH or ribose-5-phosphate.
Clinical Significance
- G6PD Deficiency: Common genetic disorder affecting NADPH production, leading to oxidative stress in red blood cells.
- Cancer: Increased PPP activity supports nucleotide production and combats oxidative stress.
Summary of the Pentose Phosphate Pathway
- Generates NADPH and ribose-5-phosphate.
- Two phases: oxidative (irreversible) and non-oxidative (reversible).
- Key enzymes: Glucose-6-phosphate dehydrogenase, transketolase, and transaldolase.
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Description
This quiz covers the essential phases of glycolysis, detailing the conversion of glucose to pyruvate, and the associated ATP and NADH production. Test your understanding of the energy investment and payoff phases, along with key enzymes involved in the process.