ch 18, 19

CH 18

18.1

  • Pyruvate dehydrogenase complex connects glycolysis and citric acid cycle.

  • Glycolysis occurs in cytoplasm; citric acid cycle occurs in mitochondria.

  • Pyruvate is converted to acetyl CoA in mitochondrial matrix via oxidative decarboxylation.

  • Links glycolysis to citric acid cycle, committing carbon to oxidation or fatty acid synthesis.

  • Reaction produces NADH, indicating electron transfer potential for citric acid cycle.

  • Pyruvate dehydrogenase complex consists of three enzymes with distinct active sites:

    • Pyruvate dehydrogenase (TPP prosthetic group) - catalyzes decarboxylation.

    • Dihydrolipoyl transacetylase (Lipoamide prosthetic group) - transfers acetyl group to CoA.

    • Dihydrolipoyl dehydrogenase (FAD prosthetic group) - regenerates oxidized lipoamide.

  • Reaction occurs in three steps:

    1. Decarboxylation to hydroxyethyl-TPP (rate-limiting step).

    2. Oxidation, forming acetyl–lipoamide and thioester bond.

    3. Formation of acetyl CoA from acetyl–lipoamide.

  • The flexible lipoamide arm facilitates substrate transfer between active sites.

  • Intermediates remain bound, enhancing reaction efficiency and minimizing side reactions.

18.2

  • The pyruvate dehydrogenase complex is stringently regulated by allosteric interactions and covalent modifications.

  • Formation of acetyl CoA from pyruvate is irreversible, preventing conversion back to glucose.

  • Acetyl CoA commits carbon from glucose for two fates:

    • Oxidation via the citric acid cycle for energy generation.

    • Incorporation into fatty acids, as acetyl CoA is crucial for fatty acid synthesis.

  • High concentrations of reaction products inhibit the pyruvate dehydrogenase complex:

    • Acetyl CoA inhibits the transacetylase component by direct binding.

    • NADH inhibits dihydrolipoyl dehydrogenase, signaling energy needs are met.

  • Covalent modification via phosphorylation regulates the complex:

    • Pyruvate dehydrogenase kinase phosphorylates and inactivates the complex.

    • Pyruvate dehydrogenase phosphatase removes the phosphoryl group to activate the complex.

  • Energy charge influences activity:

    • High acetyl CoA/CoA and ATP/ADP ratios stimulate kinase, leading to deactivation.

    • Increased ADP and pyruvate levels during muscle contraction activate the dehydrogenase by inhibiting the kinase.

  • Hormonal regulation occurs in tissues:

    • In the liver, epinephrine activates the phosphatase pathway.

    • Insulin stimulates phosphatase in tissues capable of fatty acid synthesis.

  • Defective regulation can lead to lactic acidosis due to persistent pyruvate conversion to lactate instead of acetyl CoA.

  • Cancer cells often exhibit enhanced pyruvate dehydrogenase kinase activity, promoting aerobic glycolysis (Warburg effect).

  • Thiamine pyrophosphate deficiency, a key coenzyme, can result in

  • disorders like beriberi, affecting neurological and cardiovascular systems.

CH 19

19.1

  • The citric acid cycle consists of two stages: introduction of acetyl and regeneration of oxaloacetate.

  • The cycle begins with a two-carbon acetyl unit condensing with four-carbon oxaloacetate to form six-carbon citrate.

  • Citrate is oxidized, releasing high-energy electrons and leading to the formation of a four-carbon compound.

  • Two carbons leave the cycle as carbon dioxide while regenerating oxaloacetate for another cycle.

  • The cycle produces high-energy electron carriers NADH and one molecule of ATP.

  • The citric acid cycle itself does not generate significant ATP nor requires oxygen directly.

  • High-energy electrons from NADH are used in oxidative phosphorylation to produce up to nine ATP molecules.

  • The cycle is a key component of cellular respiration, facilitating the capture of high-energy electrons from carbon fuels to generate energy efficiently.

19.2

  • The citric acid cycle oxidizes two carbon atoms to gather energy-rich electrons.

  • Primary catabolic purpose: energy extraction from acetyl CoA.

  • Initial condensation of four-carbon oxaloacetate with two-carbon acetyl unit yields six-carbon citrate.

  • Citrate loses two carbons in oxidative decarboxylation, producing a four-carbon molecule and two NADH.

  • Citrate Synthase catalyzes formation of citrate from oxaloacetate and acetyl CoA.

  • Hydrolysis of citryl CoA thioester drives citrate synthesis.

  • Mammalian citrate synthase operates as a dimer; binding order is oxaloacetate first, then acetyl CoA.

  • Enzyme undergoes conformational changes to create binding sites, preventing wasteful side reactions.

  • Citrate is isomerized to isocitrate for optimal structure for subsequent reactions.

  • Isomerization involves dehydration followed by hydration using aconitase enzyme.

  • Isocitrate undergoes oxidative decarboxylation to form alpha-ketoglutarate, generating NADH.

  • The conversion of isocitrate to succinyl CoA involves another oxidative decarboxylation step, analogous to pyruvate dehydrogenase reactions.

  • Overall, two carbon atoms enter the cycle, and two carbon atoms are oxidized, yielding two NADH.

19.3

  • The citric acid cycle regenerates oxaloacetate through a series of reactions starting and ending with four-carbon molecules.

  • Energy is harvested in the form of high-energy electron carriers and one molecule of ATP.

  • Succinyl CoA, an energy-rich thioester compound, is produced in the cycle.

  • Cleavage of the thioester from succinyl CoA drives the synthesis of citrate from oxaloacetate and a two-carbon fragment.

  • Succinyl CoA synthetase catalyzes a reaction yielding high phosphoryl-transfer potential, coupled to ADP phosphorylation.

  • Two enzyme forms exist in mammals: one for ADP, common in cells involved in respiration, and another for GDP, prevalent in liver cells performing anabolic reactions.

  • The enzyme mechanism involves displacement of coenzyme A, formation of succinyl phosphate, and ATP generation via substrate-level phosphorylation.

  • Succinate is oxidized to regenerate oxaloacetate, comprising three steps: oxidation, hydration, and a second oxidation.

  • Succinate dehydrogenase catalyzes the first oxidation step, utilizing FAD as the hydrogen acceptor.

  • Oxidation and hydration reactions continue until malate is oxidized to regenerate oxaloacetate, with NADH serving as the hydrogen acceptor in the final step.

  • The cycle produces high-energy electrons in the form of NADH, one molecule of ATP, and carbon dioxide.

  • Each complete cycle generates three NADH, one FADH2, and one ATP, yielding a net total of 10 ATP through electron transport chain processes.

19.4

  • The citric acid cycle (CAC) is the final pathway for aerobic oxidation of fuel molecules and is crucial for biosynthesis.

  • The cycle's entry and rate are controlled at multiple stages, primarily by enzymes such as isocitrate dehydrogenase and dehydrogenase.

  • Pyruvate dehydrogenase links glycolysis to the CAC, converting glucose-derived pyruvate to acetyl CoA, which can also come from fat breakdown.

  • Regulation occurs primarily through the concentrations of ATP and NADH, with isocitrate dehydrogenase activated by ADP and inhibited by NADH and ATP.

  • Dehydrogenase catalyzes a rate-limiting step and is inhibited by succinyl CoA and NADH as well as high ATP levels.

  • Isocitrate and dehydrogenase control points integrate the CAC with other pathways, affecting metabolic processes.

  • Citrate accumulation signals phosphofructokinase to halt glycolysis and serves as a source of acetyl CoA for fatty acid synthesis.

  • The CAC also serves as a source of biosynthetic precursors, with intermediates utilized for the formation of amino acids, porphyrins, and other compounds.

  • Oxaloacetate needs replenishment, especially during high energy charge states, as it's essential for the CAC to function efficiently.

  • The enzyme pyruvate carboxylase catalyzes the formation of oxaloacetate from pyruvate, an important anaplerotic reaction.

  • Defects in citric acid cycle enzymes can contribute to cancer, highlighting the cycle's role not only in energy production but in metabolic regulation as well.

19.5

  • Acetyl CoA primarily undergoes oxidation in the citric acid cycle and cannot be converted back to glucose in most organisms due to two decarboxylation steps.

  • Plants and some microorganisms can convert acetyl CoA into glucose through the glyoxylate cycle.

  • The glyoxylate cycle resembles the citric acid cycle but bypasses the two decarboxylation steps, allowing for glucose synthesis.

  • In the glyoxylate cycle, two molecules of acetyl CoA enter per turn compared to one in the citric acid cycle.

  • The cycle begins with the condensation of acetyl CoA and oxaloacetate to form citrate, which is isomerized to isocitrate.

  • Instead of decarboxylating, isocitrate is cleaved into succinate and glyoxylate by isocitrate lyase.

  • Oxaloacetate is regenerated from glyoxylate with the involvement of malate and malate synthase.

  • The glyoxylate cycle enables plants to utilize acetate for growth, especially in oil-rich seeds like sunflowers, cucumbers, and castor beans.

  • Succinate produced in this cycle can be converted into carbohydrates through the citric acid cycle and gluconeogenesis, which support seedling growth until photosynthesis begins.

  • This cycle provides metabolic flexibility, allowing organisms to use acetyl CoA as a precursor for glucose and other biomolecules.