Harper's Biochemistry Chapter 30 - Conversion of Amino Acids to Specialized Products

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Questions and Answers

What is the primary function of glycine in drug metabolism?

  • It is a precursor for neurotransmitter synthesis.
  • It acts as a neurotransmitter inhibitor.
  • It enhances the fat solubility of drugs.
  • It facilitates the formation of drug conjugates for excretion. (correct)

Which amino acids play critical roles in the biosynthesis of creatine?

  • Glycine and Threonine
  • Serine and Tyrosine
  • Arginine and S-adenosylmethionine (correct)
  • Cysteine and Histidine

Which of the following compounds is NOT formed from the decarboxylation of an amino acid?

  • Spermidine
  • Creatine (correct)
  • Histamine
  • GABA

Which amino acid contributes its carbon skeleton to the biosynthesis of polyamines?

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

What is the primary metabolic function of creatine phosphate in muscle tissue?

<p>Major energy reserve (B)</p> Signup and view all the answers

Which process describes the metabolism of β-alanine?

<p>Stepwise conversion to succinyl-CoA (A)</p> Signup and view all the answers

Which of the following roles does tryptophan NOT participate in?

<p>Direct conversion to GABA (B)</p> Signup and view all the answers

In the context of creatine metabolism, which amino acid is directly involved in its synthesis?

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

What does the excretion of creatinine in urine indicate about an individual's metabolic state?

<p>Proportional muscle mass (D)</p> Signup and view all the answers

In porphyrin biosynthesis, which intermediates are crucial for initiating the biosynthetic pathway?

<p>Acetyl-CoA and Succinyl-CoA (A)</p> Signup and view all the answers

What is the regulated step of hepatic heme biosynthesis catalyzed by?

<p>Aminolevulinic acid synthase (B)</p> Signup and view all the answers

Which metabolic compound is a direct product of the degradation of β-aminoisobutyrate?

<p>Succinyl-CoA (B)</p> Signup and view all the answers

What role do glycine and arginine play in human amino acid disorders?

<p>They are involved in creatine metabolism. (D)</p> Signup and view all the answers

What role does γ-carboxyglutamate play in biochemical processes?

<p>Facilitates calcium binding (B)</p> Signup and view all the answers

Which precursor is utilized by arginine in the synthesis of nitric oxide?

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

In the metabolism of proline, which product is formed through its hydroxylation?

<p>4-hydroxyproline (A), 3-hydroxyproline (B)</p> Signup and view all the answers

What is the primary function of creatine synthesized from arginine?

<p>Energy carrier in ATP synthesis (C)</p> Signup and view all the answers

What is a common metabolic dysfunction associated with the metabolism of cysteine?

<p>Deficiency in coenzyme A synthesis (C)</p> Signup and view all the answers

Which amino acid is primarily responsible for the incorporation of nitrogen into the urea cycle?

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

How does the metabolic pathway related to hydroxyproline affect collagen stabilization?

<p>Directly contributes to cross-linking (A)</p> Signup and view all the answers

What role does histamine play in biological systems?

<p>Regulates inflammatory responses (D)</p> Signup and view all the answers

What metabolite is formed when proline is hydroxylated in collagen biosynthesis?

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

Which amino acid serves as a precursor for the synthesis of purines and pyrimidines?

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

What metabolic consequence results from a deficiency in dihydropyrimidine dehydrogenase?

<p>Accumulation of uracil (D)</p> Signup and view all the answers

Which amino acid is directly formed from the decarboxylation of glutamate?

<p>γ-Aminobutyrate (C)</p> Signup and view all the answers

What is the primary role of β-alanyl dipeptides such as carnosine in muscle physiology?

<p>Activation of myosin APase (A)</p> Signup and view all the answers

What is one of the metabolic products of transamination of GABA?

<p>Succinate semialdehyde (B)</p> Signup and view all the answers

Which of the following is formed from the catabolism of β-aminoisobutyrate?

<p>Acetyl-CoA (A)</p> Signup and view all the answers

Which intermediate is directly involved in the conversion of methymalonate semialdehyde?

<p>Succinyl-CoA (B)</p> Signup and view all the answers

Which disorder is linked to dysfunction in the enzyme involved in β-alanine metabolism?

<p>Dihydropyrimidine dehydrogenase deficiency (C)</p> Signup and view all the answers

What process primarily describes the metabolic conversion of uracil?

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

Which compound is produced by the reduction of succinate semialdehyde?

<p>γ-Hydroxybutyrate (A)</p> Signup and view all the answers

What is a potential consequence of increased β-alanine levels in the body?

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

What is the primary effect of administering iproniazid on serotonin levels?

<p>It prolongs the action of serotonin. (A)</p> Signup and view all the answers

Which urinary metabolite is indicative of carcinoid tumor activity?

<p>N-acetylserotonin glucuronide (B)</p> Signup and view all the answers

What is the consequence of serotonin N-acetylation followed by O-methylation?

<p>Conversion to melatonin. (C)</p> Signup and view all the answers

Which metabolic process is responsible for converting tryptophan in kidney tissue?

<p>Conversion to tryptamine, and then to indole 3-acetate. (D)</p> Signup and view all the answers

How are serotonin and 5-methoxytryptamine primarily broken down in the body?

<p>Metabolized to corresponding acids by monoamine oxidase. (A)</p> Signup and view all the answers

What is the main consequence of tryptophan metabolism in the context of melatonin production?

<p>It enhances the conversion of serotonin to melatonin. (C)</p> Signup and view all the answers

What is the key enzyme involved in the synthesis of epinephrine and norepinephrine from tyrosine?

<p>L-aromatic amino acid decarboxylase. (B)</p> Signup and view all the answers

What major urinary catabolite is commonly associated with tryptophan breakdown?

<p>5-hydroxyindoleacetate. (B)</p> Signup and view all the answers

Which of the following tissues have the capacity to convert tryptophan into tryptamine?

<p>Liver and kidney tissues. (C)</p> Signup and view all the answers

What is the role of gluconeogenesis regarding amino acids in metabolism?

<p>It synthesizes glucose from non-carbohydrate sources like amino acids. (D)</p> Signup and view all the answers

What is the function of γ-carboxyglutamate in the body?

<p>It facilitates calcium ion binding. (B)</p> Signup and view all the answers

Which amino acid conversion is primarily associated with the stabilization of collagen fibers?

<p>Hydroxylation of proline to form hydroxyproline. (D)</p> Signup and view all the answers

Which compound is produced during the metabolic conversion of arginine?

<p>Nitric oxide (B)</p> Signup and view all the answers

Which amino acid primarily contributes to the synthesis of neurotransmitters?

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

How does cysteine contribute to cellular metabolism?

<p>It is involved in the formation of coenzyme A. (C)</p> Signup and view all the answers

What role does nitric oxide serve in physiological processes?

<p>It functions as a signaling molecule and vasodilator. (B)</p> Signup and view all the answers

What metabolic pathway is directly associated with the conversion of arginine into putrescine?

<p>The polyamine biosynthetic pathway. (C)</p> Signup and view all the answers

What is the reaction catalyzed by sarcosine dehydrogenase?

<p>Sarcosine + FAD + H4PG + H2O → Glycine + FADH2 (C)</p> Signup and view all the answers

Which of the following metabolites is directly formed from creatine phosphate in muscle tissue?

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

Which amino acids contribute to the biosynthesis of creatine?

<p>Methionine and Arginine (A)</p> Signup and view all the answers

The catabolism of β-alanine primarily starts with which biochemical process?

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

What is the role of S-adenosylmethionine in the synthesis of creatine?

<p>It serves as a methyl donor for the conversion of guanidoacetate. (B)</p> Signup and view all the answers

What is the primary action of iproniazid in relation to serotonin?

<p>It inhibits the breakdown of serotonin. (D)</p> Signup and view all the answers

Which urinary metabolite is primarily produced from serotonin metabolism?

<p>N-acetylserotonin glucuronide (A)</p> Signup and view all the answers

What is the metabolic fate of circulating melatonin in the body?

<p>It is conjugated with glucuronic acid or sulfate. (C)</p> Signup and view all the answers

Which of the following enzymes is involved in the conversion of tyrosine to norepinephrine?

<p>Dopamine β-hydroxylase (B)</p> Signup and view all the answers

Which compound is a primary catabolite of tryptophan in urine?

<p>Indole-3-acetate (D)</p> Signup and view all the answers

Which metabolic pathway is primarily responsible for the degradation of serotonin and related compounds?

<p>Monoamine oxidase pathway (D)</p> Signup and view all the answers

Which of the following statements is true regarding the metabolism of tryptophan in various tissues?

<p>Tryptophan can be metabolized to tryptamine in kidney and liver tissues. (A)</p> Signup and view all the answers

What is the role of monoamine oxidase in the metabolism of serotonin?

<p>It initiates the breakdown of serotonin into its metabolites. (D)</p> Signup and view all the answers

Which tissue has the capacity to convert both tryptophan and serotonin into metabolites?

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

What is the significance of N-acetylation of serotonin followed by O-methylation?

<p>It generates melatonin for physiological functions. (A)</p> Signup and view all the answers

What is the function of DOPA decarboxylase in the synthesis of neurotransmitters?

<p>It catalyzes the decarboxylation of DOPA to produce dopamine. (B)</p> Signup and view all the answers

Which enzyme is responsible for the conversion of norepinephrine to epinephrine in the adrenal medulla?

<p>Phenylethanolamine N-methyltransferase (D)</p> Signup and view all the answers

In serotonin biosynthesis, which compound is a precursor in the catecholamine pathway?

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

Which of the following describes the role of serine in metabolism?

<p>It donates carbons for the synthesis of sphingosine and pyrimidines. (C)</p> Signup and view all the answers

What is the outcome of the reaction catalyzed by cystathionine β-synthase?

<p>Conversion of homocysteine to cystathionine using serine. (C)</p> Signup and view all the answers

Which step in the neurotransmitter synthesis pathway does dopamine β-oxidase catalyze?

<p>Hydroxylation of dopamine to yield norepinephrine. (D)</p> Signup and view all the answers

What metabolic pathway is linked to the formation of sarcosine?

<p>Biosynthesis and catabolism occurring in mitochondria. (D)</p> Signup and view all the answers

Which of the following roles does tyrosine NOT participate in?

<p>Direct contributor to purine bases. (D)</p> Signup and view all the answers

What is the consequence of a defect in cystathionine β-synthase?

<p>Accumulation of homocysteine leading to homocystinuria. (A)</p> Signup and view all the answers

Which of the following statements about glycine conjugation is true?

<p>Conjugation with glycine facilitates the elimination of drugs through urine. (D)</p> Signup and view all the answers

What is the effect of histidine decarboxylation on the body?

<p>It increases the levels of histamine, influencing allergic reactions. (A)</p> Signup and view all the answers

In which of the following conditions would urinary levels of 3-methylhistidine be expected to be unusually low?

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

What important role does glycine play in the formation of heme?

<p>It supplies atoms to the pyrrole rings of heme. (D)</p> Signup and view all the answers

Which of the following compounds is primarily associated with excitatory tissues in the human body?

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

Which of the following statements correctly describes the relationship between glycine and benzoate?

<p>Glycine is a conjugate of hippuric acid formed from benzoate. (C)</p> Signup and view all the answers

What would most likely be the result of the decarboxylation of histidine?

<p>Formation of histamine in the body. (A)</p> Signup and view all the answers

Which amino acid is found to supply crucial atoms in the synthesis of purine bases?

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

How does glycine conjugation impact the solubility of metabolites?

<p>It significantly increases their water solubility. (C)</p> Signup and view all the answers

Match the following amino acids with their primary metabolic roles:

<p>Alanine = Carrier of ammonia and pyruvate Arginine = Precursor for nitric oxide synthesis Cysteine = Biosynthesis of coenzyme A Proline = Formation of hydroxyproline in collagen</p> Signup and view all the answers

Match the following neurotransmitters with their respective functions:

<p>Histamine = Regulator of physiological processes Nitric Oxide = Smooth muscle relaxant GABA = Inhibitory neurotransmitter Dopamine = Regulates mood and movement</p> Signup and view all the answers

Match the following compounds with their respective precursors:

<p>Creatine = Arginine Putrescine = Ornithine Heme = Glycine Glutathione = Cysteine</p> Signup and view all the answers

Match the following metabolic processes with their functions:

<p>Urea Cycle = Nitrogen elimination Collagen Hydroxylation = Stabilization of collagen fibers Beta-Oxidation = Fatty acid degradation Transamination = Amino acid conversion</p> Signup and view all the answers

Match the following amino acids with their contribution to metabolic pathways:

<p>Glycine = Precursor for purines Proline = Formation of hydroxyproline Arginine = Source of nitrogen in urea Alanine = Transport of nitrogen to the liver</p> Signup and view all the answers

Match the polyamines with their primary biosynthetic precursors:

<p>Spermidine = Decarboxylated S-adenosylmethionine Spermine = Spermidine Putrescine = Ornithine S-adenosylmethionine = Methionine</p> Signup and view all the answers

Match the enzymes with their associated reactions:

<p>Ornithine decarboxylase = Formation of Putrescine Spermidine synthase = Formation of Spermidine Spermine synthase = Formation of Spermine Methionine adenosyltransferase = Biosynthesis of S-adenosylmethionine</p> Signup and view all the answers

Match the compounds with their structural components:

<p>S-adenosylmethionine = Methionine + Adenine Putrescine = Ornithine + decarboxylation Spermidine = Putrescine + Aminopropyl transfer Spermine = Spermidine + Aminopropyl transfer</p> Signup and view all the answers

Match the following with their biological roles:

<p>S-adenosylmethionine = Methyl donor in various reactions Putrescine = Induces cell proliferation and growth Spermidine = Stabilizes cellular processes Spermine = Protects against cellular damage</p> Signup and view all the answers

Match the polyamines with their pharmacological effects:

<p>Polyamines = Hypothermic and hypotensive effects Spermidine = Enhances cellular stability Spermine = Inhibits cellular stress Putrescine = Promotes cellular growth</p> Signup and view all the answers

Flashcards

Połyamines metabolism

The process of breaking down polyamines, which are important for metabolic regulation including signal transduction.

Peptide-bound amino acids

Serine, threonine, and tyrosine are modified forms, and play roles in metabolic regulation and signal transduction.

Creatine biosynthesis

The creation of creatine, a crucial energy reserve in muscles and brain, made from glycine, arginine, and S-adenosylmethionine.

Creatine phosphate

A crucial energy storage molecule, part of the creatine system.

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Creatinine excretion

The removal of creatinine, a breakdown product of creatine, in urine, proportional to muscle mass.

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β-Alanine

A free amino acid found in tissues, and part of coenzyme A, its breakdown leads to acetyl-CoA.

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β-Alanine catabolism

The process of breaking down β-alanine to acety-CoA.

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β-Aminoisobutyrate

Another free amino acid that is broken down to succinyl-CoA.

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Heme biosynthesis

The process that creates heme, a crucial component of hemoglobin.

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Amphibolic intermediates

Key molecules involved in the start of heme synthesis.

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Key regulated step of hepatic heme biosynthesis

A critical step in heme synthesis, which is controlled by the liver.

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Serotonin Metabolism

Serotonin is metabolized by monoamine oxidase to produce various metabolites, including N-acetylated serotonin, 5-hydroxyindoleacetate.

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Carcinoid Tumor

A tumor characterized by excessive serotonin production, leading to increased urinary metabolites like N-acetylated serotonin glucuronide and 5-hydroxyindoleacetate glycine conjugate.

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Tryptophan Metabolism

Tryptophan is broken down to tryptamine and indole 3-acetate, producing 5-hydroxyindoleacetate, important urinary metabolites.

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Tyrosine Conversion

Neurons convert tyrosine into epinephrine and norepinephrine.

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Melatonin Synthesis

Melatonin is produced from serotonin through acetylation and methylation reactions in the pineal gland.

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Melatonin Metabolism

Melatonin circulating in the bloodstream is taken up by tissues, including the brain, and rapidly metabolized through hydroxylation followed by conjugation with sulfate or glucuronic acid.

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Monoamine Oxidase

An enzyme that catalyzes the breakdown of serotonin and other monoamines.

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Amino Acid Metabolism

The process where amino acids are converted into various molecules, like heme, purines, pyrimidines, hormones, neurotransmitters, and bioactive peptides.

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γ-carboxyglutamate

A modified form of glutamate, important for calcium binding.

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Hydroxyproline

A modified form of proline, crucial for collagen formation.

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5-hydroxylysine

A modified form of lysine, crucial for collagen stability.

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Cori Cycle

The cycle that transports ammonia and carbons from skeletal muscle to the liver.

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Arginine metabolism

The process of breaking down Arginine into various molecules, including creatine, nitric oxide, ornithine, putrescine and spermine.

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Nitric Oxide

A signaling molecule produced from arginine, involved in muscle relaxation and vasodilation.

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Coenzyme A

A molecule involved in beta-alanine breakdown to acetyl-CoA

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β-Alanine catabolism

The breakdown of β-alanine into acetyl-CoA.

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β-Aminoisobutyrate

An amino acid that is broken down into succinyl-CoA.

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β-Alanyl dipeptides

Dipeptides like carnosine and anserine, that affect muscle function and copper.

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γ-aminobutyrate (GABA)

An inhibitory neurotransmitter made from glutamate, involved in brain function

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GABA metabolism disorders

Genetic defects affecting GABA's processing, leading to various issues.

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Dihydropyrimidine dehydrogenase deficiency

A metabolic disorder affecting the breakdown of pyrimidines, leading to β-alanine and β-aminoisobutyrate issues.

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GABA neurotransmitter

A neurotransmitter formed by decarboxylation of glutamate, playing a role in inhibitory signaling.

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Glycine conjugation

Process where drugs and metabolites combine with glycine to enhance their water solubility for easier excretion.

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Histamine neurotransmitter

Neurotransmitter formed through decarboxylation of histidine, involved in various bodily functions.

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S-Adenosylmethionine (SAM)

Key source of methyl groups. Involved in the synthesis of polyamines.

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Serine's metabolic roles

Serine contributes to purine and thymine's construction and sphingosine synthesis, plus part of phospholipid structure.

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Tryptophan Catabolism

Tryptophan breaks down into tryptamine, indole-3-acetate, and 5-hydroxyindoleacetate.

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Tyrosine's roles

Forms norepinephrine, epinephrine, thyroid hormones triiodothyronine and thyroxine.

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GABA catabolism disorders

Rare metabolic conditions resulting from defects in GABA breakdown pathways.

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Tryptophan breakdown products

"Indole 3-acetate" and "5-hydroxyindoleacetate" are found in urine.

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γ-carboxyglutamate

A modified form of glutamate, crucial for calcium binding in bodily processes.

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Hydroxyproline

A modified form of proline, important for collagen formation and structure.

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5-hydroxylysine

A modified form of lysine, essential for collagen stability and cross-linking.

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Cori Cycle

Cycle transporting ammonia and carbon from muscle to liver.

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Arginine metabolism

Breaking down Arginine into various molecules, including creatine, nitric oxide, ornithine, putrescine, and spermine.

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Nitric Oxide

A signaling molecule made from arginine, involved in muscle relaxation and vasodilation.

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Coenzyme A

Molecule involved in beta-alanine breakdown into acetyl-CoA.

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β-Alanine catabolism

The breakdown of β-alanine into acetyl-CoA.

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β-Aminoisobutyrate

An amino acid broken down into succinyl-CoA.

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Amino Acid Metabolism

The process of converting amino acids into various substances like heme, purines, and neurotransmitters.

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Serotonin Metabolism

The breakdown of serotonin by monoamine oxidase into various metabolites, including N-acetylated serotonin and 5-hydroxyindoleacetate.

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Carcinoid Tumor

A tumor that overproduces serotonin, leading to increased urinary levels of specific serotonin metabolites.

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Tryptophan Metabolism

The breakdown of tryptophan into tryptamine, indole-3-acetate, and 5-hydroxyindoleacetate.

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Tyrosine Conversion

Neurons convert tyrosine into epinephrine and norepinephrine.

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Melatonin Synthesis

Melatonin is produced from serotonin through acetylation and methylation reactions in the pineal gland.

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Melatonin Metabolism

Circulating melatonin is absorbed by tissues (like the brain) and metabolized through hydroxylation, followed by conjugation with sulfate or glucuronic acid.

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Monoamine Oxidase

An enzyme that breaks down serotonin and other monoamine neurotransmitters.

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Amino Acid Metabolism

The process where amino acids are converted into various molecules, including heme, purines, pyrimidines, hormones, neurotransmitters, and bioactive peptides.

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Analogous Reactions

Acidic drugs and catabolites often react similarly.

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Glycine Conjugates

Water-soluble molecules formed by combining glycine with other molecules.

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Hippuric Acid

A glycine conjugate formed from a food additive, benzoate.

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Histidine Decarboxylation

Conversion of histidine to histamine by an enzyme.

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Histamine

A biogenic amine involved in allergic reactions and stomach acid secretion.

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Carnosine/Homocarnosine

Major components of muscles and brain tissues; histidine-derived dipeptides.

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Ergothioneine

A histidine-containing compound found in the human body, derived from cysteine

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Glycine's Role in Purine Bases

Glycine provides atoms 4, 5, and 7 in the structure of purine bases.

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Glycine's Role in Creatine Synthesis

Glycine is a component of creatine, in which its nitrogen and α-carbon are components of the structure.

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Glycine Conjugation for Excretion

Glycine conjugation makes compounds more water soluble, aiding in their excretion in urine.

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Dopamine formation

Tyrosine is hydroxylated, then decarboxylated to form dopamine.

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Norepinephrine formation

Dopamine is hydroxylated to form norepinephrine.

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Epinephrine formation

Norepinephrine is methylated by phenylethanolamine N-methyltransferase to produce epinephrine.

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Tyrosine Precursor

Tyrosine is a precursor to thyroid hormones triiodothyronine and thyroxine.

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Serine roles

Serine is vital for sphingosine synthesis & part of the structure of phospholipids and also involved in the synthesis of purines and pyrimidines.

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Cystathionine formation

Homocysteine and serine combine to form cystathionine via cystathionine β-synthase.

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Serine + Homocysteine

Serine and homocysteine react to produce cystathionine with water as a byproduct.

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Phosphorylation/Dephosphorylation

Phosphorylation & dephosphorylation of amino acids regulate enzymes and protein function, involved in the regulation of lipid and carbohydrate metabolism and signal transduction.

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Sarcosine catabolism

The breakdown of sarcosine to glycine, catalyzed by sarcosine dehydrogenase.

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Creatine phosphate to Creatinine

Creatine phosphate is converted to creatinine through a non-enzymatic dehydration reaction, releasing phosphate

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Creatinine excretion

The removal of creatinine from the body in urine, reflecting muscle mass.

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Creatine biosynthesis

The formation of creatine, a compound crucial for energy storage in muscle cells, from glycine, arginine, and S-adenosylmethionine.

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Sarcosine

A non-proteinogenic amino acid derivative derived from glycine and a methyl group

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β-alanine

A non-proteinogenic amino acid found in tissues and in specific dipeptides that affects muscle function and copper metabolism

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β-Aminoisobutyrate

A non-proteinogenic amino acid that is derived from the breakdown of valine and involved in various metabolic processes.

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Sarcosine dehydrogenase

Enzyme that catalyzes the conversion of sarcosine to glycine

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One-carbon units

Methyl groups that are released from sarcosine when undergoing catabolism and are important for metabolic pathways

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S-Adenosylmethionine (SAM)

A methyl donor crucial for various metabolic reactions, including creatine synthesis.

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S-Adenosylmethionine (SAM)

A key source of methyl groups, involved in the synthesis of polyamines.

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Polyamines

Molecules functioning in cell proliferation, growth, and stabilizing cells and subcellular structures.

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Polyamine Biosynthesis

The creation of polyamines from methionine and ornithine.

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Polyamine Catabolism

The breakdown of polyamines.

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Creatine Biosynthesis

The process of creating creatine from glycine, arginine, and S-adenosylmethionine.

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Creatine Phosphate

A crucial energy storage molecule used in the creatine system.

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Creatinine Excretion

The removal of creatinine, a breakdown product of creatine, from the body in urine.

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Amino Acid Metabolism

Process where amino acids are converted to many essential molecules.

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Serine's Metabolic Roles

Serine is involved in purine and thymine synthesis, sphingosine synthesis and is part of phospholipid structure.

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Serine + Homocysteine

Combines to form cystathionine.

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γ-carboxyglutamate

A modified form of glutamate, importantly involved in calcium binding.

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Hydroxyproline

A modified form of proline, crucial for collagen's structure.

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5-hydroxylysine

A modified form of lysine, vital for collagen stability.

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Cori Cycle

Cycle transferring ammonia and carbons between muscle and liver.

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Arginine metabolism

Breakdown of arginine creating creatine, nitric oxide, ornithine, putrescine, and spermine.

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Nitric Oxide

A signaling molecule from arginine, relating to muscle relaxation and vasodilation.

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Coenzyme A

Molecule involved in beta-alanine breakdown into acetyl-CoA.

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β-Alanine catabolism

Breakdown of beta-alanine into acetyl-CoA.

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β-Aminoisobutyrate

Amino acid broken down into succinyl-CoA.

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Amino Acid Metabolism

Conversion of amino acids into various molecules, like heme, purines, and neurotransmitters.

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Serotonin Metabolism

Breakdown of serotonin by monoamine oxidase into various metabolites.

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Carcinoid Tumor

Tumor overproducing serotonin, leading to increased urinary serotonin metabolites.

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Tryptophan Metabolism

Breakdown of tryptophan into tryptamine, indole-3-acetate, and 5-hydroxyindoleacetate.

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Tyrosine Conversion

Conversion of tyrosine into epinephrine and norepinephrine in neurons.

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Melatonin Synthesis

Production of melatonin from serotonin via acetylation and methylation.

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Melatonin Metabolism

Breakdown of circulating melatonin by hydroxylation and conjugation in tissues.

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Study Notes

Conversion of Amino Acids to Specialized Products

  • Amino acids participate in various biosynthetic processes beyond protein synthesis.
  • Arginine is involved in the biosynthesis of creatine, nitric oxide (NO), putrescine, spermidine, and spermine.
  • Cysteine and β-alanine contribute to coenzyme A structure.
  • Glycine plays a role in drug catabolism and excretion, along with heme, purines, creatine, and sarcosine synthesis.
  • S-adenosylmethionine is crucial in metabolism.
  • Tryptophan metabolites include serotonin, melatonin, tryptamine, and indole 3-acetate.
  • Tyrosine is a precursor for norepinephrine and epinephrine.
  • Peptidyl serine, threonine, and tyrosine are key in metabolic regulation and signal transduction.
  • Creatine phosphate is essential for energy homeostasis.
  • γ-aminobutyrate (GABA) biosynthesis and related metabolic disorders are discussed.

Biomedical Importance

  • Certain amino acids undergo post-translational modifications for specific functions.
  • Examples include carboxylation of glutamate (Ca²+ binding), hydroxylation of proline and lysine (collagen), and these modifications stabilize collagen fibers.
  • Amino acids are precursors for various crucial biological materials like heme, purines, pyrimidines, hormones, neurotransmitters, and biologically active peptides.
  • Histamine plays a role in allergic reactions.
  • Neurotransmitters like γ-aminobutyrate (GABA), serotonin, dopamine, norepinephrine, and epinephrine are derived from amino acids.
  • Many drugs affect neurotransmitter metabolism.

L-α-Amino Acids

  • Alanine transports ammonia and pyruvate carbons between muscle and liver.

Arginine

  • Arginine is part of urea biosynthesis and creatine, putrescine, and spermine synthesis.
  • It's crucial in nitric oxide synthesis and smooth muscle regulation.

Cysteine

  • Cysteine contributes to coenzyme A biosynthesis.
  • Also, is involved in taurine and bile acid formation.

Additional Information

  • Figures 30-1 to 30-13, and Figures 31-1 and 31-2 are relevant to understanding specific metabolic pathways.
  • Many amino acid pathways are interconnected, and the notes highlight some of these relationships.
  • Important enzymes are mentioned for each pathway.
  • Some deficiencies and disorders are also discussed, including homocystinuria, Wilson's disease, and those related to GABA catabolism.

Creatine and Creatinine

  • Creatine is derived from glycine, arginine, and methionine.
  • Creatine phosphate is vital for muscle energy.
  • Creatinine levels reflect muscle mass.
  • Creatinine is a byproduct of creatine phosphate breakdown.

Porphyrins and Bile Pigments

  • Porphyrins are cyclic compounds with four pyrrole rings.
  • Porphyrins are involved in heme synthesis.
  • Heme plays roles in oxygen transport, storage, and electron transport.
  • Porphyrias are diseases resulting from defects in porphyrin synthesis.
  • Jaundice is an elevated bilirubin level, often resulting from issues with heme breakdown.
  • "Direct bilirubin" and "indirect bilirubin" are terms used to describe certain forms of bilirubin.

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