Pentose Phosphate Pathway Overview
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Questions and Answers

What is the primary regulation point of the pentose phosphate pathway?

  • Transaldolase reaction
  • Ribulose 5-phosphate isomerase
  • Glucose 6-phosphate dehydrogenase reaction (correct)
  • Fructose 1,6-bisphosphate

What role does insulin play in the regulation of the pentose phosphate pathway?

  • It has no effect on G6PD expression.
  • It up regulates the expression of G6PD. (correct)
  • It inhibits the expression of G6PD.
  • It down regulates G6PD in fasted states.

What is a major consequence of G6PD deficiency?

  • Increased detoxification of oxidizing agents
  • Increased resistance to bacterial infections
  • Enhanced formation of NADPH
  • Hemolytic anemia (correct)

How is G6PD deficiency inherited?

<p>X-linked inheritance (B)</p> Signup and view all the answers

What is a common clinical manifestation of G6PD deficiency in newborns?

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

What evolutionary advantage is associated with G6PD deficiency?

<p>Resistance to falciparum malaria (C)</p> Signup and view all the answers

What must be balanced against the risks of hemolytic anemia in G6PD deficiency?

<p>Resistance to certain pathogens (B)</p> Signup and view all the answers

What is a primary outcome of the pentose phosphate pathway?

<p>Formation of NADPH (D)</p> Signup and view all the answers

Which cell types are particularly active in NADPH-dependent fatty acid biosynthesis?

<p>Liver and adipose tissue (B)</p> Signup and view all the answers

What does glucose 6-phosphate dehydrogenase (G6PD) perform in the pentose phosphate pathway?

<p>Irreversible oxidation of glucose 6-phosphate (A)</p> Signup and view all the answers

Which of the following compounds is produced in the oxidative phase of the pentose phosphate pathway?

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

What type of reactions occur in the non-oxidative phase of the pentose phosphate pathway?

<p>Reversible sugar-phosphate interconversions (C)</p> Signup and view all the answers

Which biochemical role does NADPH serve in the body?

<p>A reducing agent in anabolic reactions (A)</p> Signup and view all the answers

Glucose 6-phosphate dehydrogenase deficiency can lead to which of the following consequences?

<p>Higher levels of reactive oxygen species (D)</p> Signup and view all the answers

In which organ is the pentose phosphate pathway particularly crucial for synthesizing steroid hormones?

<p>Adrenal cortex (A)</p> Signup and view all the answers

Flashcards

Pentose Phosphate Pathway

A metabolic pathway that generates NADPH and ribose 5-phosphate, important for biosynthesis and reducing power.

NADPH function

Acts as a biochemical reductant, crucial for biosynthesis, especially fatty acids and steroid hormones.

Ribose 5-phosphate

A crucial component needed for nucleotide biosynthesis.

Glucose 6-Phosphate Dehydrogenase (G6PD)

Enzyme catalyzing the initial, irreversible oxidative step in the pentose phosphate pathway.

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Glucose 6-phosphate deficiency effect

Leads to reduced NADPH production, which weakens the body's defense against oxidative stress, particularly in red blood cells.

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NADPH importance in RBCs

Keeps glutathione reduced, protecting red blood cells from oxidative damage.

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Irreversible reactions in Pentose Phosphate Pathway

Oxidative portion forms ribulose 5-phosphate, CO2, and two NADPH, per glucose 6-phosphate oxidized molecule.

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Pentose phosphate pathway site

Occurring in the cytosol of the cell, with importance in liver, mammary glands, adipose tissue, and red blood cells.

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Pentose Phosphate Pathway Regulation

Primarily controlled at the Glucose-6-Phosphate Dehydrogenase (G6PD) reaction. Insulin upregulates G6PD gene expression, increasing pathway flux during fed states.

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G6PD Deficiency

Inherited disease causing hemolytic anemia due to inability to detoxify oxidizing agents, impairing NADPH production in red blood cells.

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G6PD Deficiency Cause

X-linked genetic disorder, stemming from over 400 different mutations in the G6PD gene.

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G6PD Deficiency Symptoms

Hemolytic anemia and Neonatal jaundice (1-4 days after birth) due to increased unconjugated bilirubin.

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G6PD Deficiency's Impact

Can shorten lifespan in severe cases due to chronic hemolysis; however, female carriers have increased malaria resistance.

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Glucose-6-Phosphate Dehydrogenase (G6PD)

Enzyme crucial in the pentose phosphate pathway, critical for detoxifying oxidizing agents and making NADPH, needed in red blood cells.

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NADPH role in RBCs

Crucial antioxidant, protecting red blood cells from damage by oxidizing agents.

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Study Notes

Pentose Phosphate Pathway

  • The pentose phosphate pathway, also called the hexose monophosphate pathway or 6-phosphogluconate pathway, occurs in the cytosol of cells.
  • The pathway involves two, irreversible oxidative reactions, followed by reversible sugar-phosphate interconversions.
  • The pathway does not directly consume or produce ATP.

Specific Objectives

  • Understand the function of the pentose shunt.
  • Explain the effect of glucose 6-P dehydrogenase deficiency.

Overview of Pentose Phosphate Pathway

  • The pathway provides the body with NADPH, a crucial biochemical reductant.
  • It produces ribose-5-phosphate needed for nucleotide biosynthesis.
  • It helps metabolize five-carbon sugars from the diet or carbohydrates in the body.

Irreversible Oxidative Reactions

  • The production of ribulose 5-phosphate, CO2, and two molecules of NADPH occur for each molecule of glucose 6-phosphate oxidized.
  • One glucose 6-phosphate oxidized results in two NADPH molecules.

Oxidative Stages of Pentose Phosphate Pathway

  • Glucose-6-phosphate undergoes dehydrogenase to 6-phosphogluconolactone. This is followed by gluconolactonase that produces 6-phosphogluconate. 6-phosphogluconate undergoes dehydrogenase to produce ribulose-5-P.
  • The reactions generate NADPH and CO2, and are crucial for reducing oxidative stress and providing NADPH for biosynthesis.

Site of Action

  • The pentose pathway is vital in the liver, lactating mammary glands, and adipose tissue, which produce fatty acids, especially those dependent on NADPH.
  • It's also key in the testes, ovaries, placenta, and adrenal cortex for steroid hormone synthesis, again dependent on NADPH.
  • Red blood cells (RBCs) require NADPH to keep glutathione reduced.

Reversible Nonoxidative Reactions

  • These reactions occur in all cells that synthesize nucleotides and nucleic acids.
  • They allow ribulose-5-phosphate to yield ribose-5-phosphate, a component of nucleotide synthesis.
  • They also generate intermediates of glycolysis (fructose 6-phosphate and glyceraldehyde 3-phosphate).

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

  • G6PD deficiency is an inherited condition causing hemolytic anemia.
  • Erythrocytes are unable to detoxify oxidative agents due to inadequate NADPH production, leading to hemolysis.
  • G6PD deficiency is X-linked, stemming from various gene mutations.

Clinical Manifestations of G6PD Deficiency

  • Neonatal jaundice, potentially severe, frequently arises 1 to 4 days after birth due to increased production of unconjugated bilirubin.
  • Chronic hemolysis can shorten the lifespan of individuals with severe G6PD deficiency.
  • Female carriers of the deficient gene show increased resistance to malaria.

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Description

Explore the key functions and processes of the pentose phosphate pathway. This quiz delves into its role in NADPH production, nucleotide biosynthesis, and the implications of glucose 6-P dehydrogenase deficiency. Test your understanding of this essential metabolic pathway.

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