Purines and Pyrimidine Metabolism Quiz
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Questions and Answers

What are the two main components of nucleotides?

  • Amino acids and fatty acids
  • Lipids and proteins
  • Sugar and phosphate groups (correct)
  • Purines and pyrimidines (correct)

What is the role of pancreatic juice in the digestion of nucleic acids?

  • It hydrolyses nucleic acids into mononucleotides (correct)
  • It absorbs nucleic acids
  • It converts nucleotides into pyrimidines
  • It synthesizes purines and pyrimidines

Which of the following is NOT a source of dietary purines and pyrimidines?

  • Liver and meat
  • Legumes and cereals
  • Tea, coffee, and cola
  • Egg, milk, and cheese (correct)

What is the importance of UDP-glucuronic acid in the body?

<p>It acts as a donor for conjugation reactions (B)</p> Signup and view all the answers

Which of the following is a biomedical importance of purines and pyrimidines?

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

What happens to ingested nucleic acids and nucleotides in the intestinal tract?

<p>They are degraded to mononucleotides (B)</p> Signup and view all the answers

What is the term for the compounds formed from the linkage of a sugar with a nitrogen-containing base?

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

Which of the following is a gout disease treatment?

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

What is the primary function of nucleosidases?

<p>Hydrolyze nucleosides into free bases and pentose (D)</p> Signup and view all the answers

What happens to unabsorbed purines in the gut?

<p>They are converted to uric acid by intestinal bacteria (A)</p> Signup and view all the answers

What is the site of purine base catabolism?

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

What is the end product of purine base catabolism in humans?

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

What is the normal range of uric acid in females?

<p>2-6mg/dl (B)</p> Signup and view all the answers

What is the underlying cause of gout?

<p>Increased production and deposition of uric acid crystals (A)</p> Signup and view all the answers

What is Lesch-Nyhan syndrome inherited as?

<p>X-linked recessive disorder (C)</p> Signup and view all the answers

What is the most painful manifestation of gout?

<p>Acute gouty arthritis (C)</p> Signup and view all the answers

What is the primary cause of Lesch-Nyhan syndrome?

<p>Deficiency of hypoxanthine-guanine phosphoribosyl transferase (A)</p> Signup and view all the answers

What is the composition of renal stones in gout?

<p>Mainly composed of uric acid and/or urates (A)</p> Signup and view all the answers

In which organisms is ammonia the end product of protein catabolism?

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

What is a common symptom of Lesch-Nyhan syndrome?

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

What is hypouricemia due to?

<p>Decreased uric acid formation (D)</p> Signup and view all the answers

What is the result of adenosine deaminase deficiency?

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

What is the daily excretion of uric acid in urine?

<p>0.7gm/day (C)</p> Signup and view all the answers

What is the effect of a purine-free diet on uric acid excretion?

<p>Decreased excretion (D)</p> Signup and view all the answers

Which of the following statements about the catabolism of pyrimidines is TRUE?

<p>β-alanine is the major end product of both uracil and cytosine. (A)</p> Signup and view all the answers

What is the main cause of β-amino isobutyric aciduria?

<p>Defective deamination of β-aminoisobutyric acid. (A)</p> Signup and view all the answers

What are the characteristic symptoms of orotic aciduria?

<p>Retarded growth, severe anemia, and leukopenia. (B)</p> Signup and view all the answers

What is the mechanism by which uridine and cytidine treat orotic aciduria?

<p>They increase UMP production, which inhibits CPS-II and reduces orotic acid production. (A)</p> Signup and view all the answers

Which of the following conditions can lead to increased levels of β-aminoisobutyric acid in urine?

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

What is the role of Orotate phosphoribosyl transferase and Orotidine 5'-phosphate decarboxylase (OMP decarboxylase) in pyrimidine metabolism?

<p>They are involved in the conversion of orotate to UMP. (C)</p> Signup and view all the answers

Which of the following statements about orotic aciduria Type I and Type II is TRUE?

<p>Orotic aciduria Type I and Type II are caused by different genetic mutations but lead to the same clinical manifestations. (D)</p> Signup and view all the answers

Which of the following is NOT a characteristic of orotic aciduria?

<p>Deficiency in purine metabolism. (B)</p> Signup and view all the answers

Flashcards

Purines and Pyrimidines

Heterocyclic aromatic compounds that, along with sugar and phosphate groups, are components of nucleotides.

Purines

Adenine and guanine, double-ring structures.

Pyrimidines

Thymine, uracil, and cytosine; single-ring structures.

Nucleoside

A sugar linked to a nitrogen-containing base.

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Purines and Pyrimidines Importance

DNA and RNA are composed of these.

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Purine/Pyrimidine Non-Essentiality

Body can synthesize; not essential in diet.

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Pancreatic Nucleases

Hydrolyze nucleic acids into mononucleotides

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Uric Acid Formation

Converted from unabsorbed purines by intestinal bacteria.

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Purine Catabolism Site

Liver (intracytoplasmic)

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Xanthine Oxidase

Enzyme that oxidizes hypoxanthine into xanthine, then to uric acid.

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Uric Acid

End product of purine base catabolism.

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Gout

Excess uric acid deposition, causing arthritis.

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Hyperuricemia

Recurrent attacks of acute inflammatory arthritis.

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Lesch-Nyhan Syndrome

An X-linked recessive disorder due to HGPRT deficiency.

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Lesch-Nyhan Cause

Deficiency of HGPRT.

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Lesch-Nyhan Symptoms

Severe gout, kidney problems, poor muscle control, mental retardation.

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Hypouricemia

Low uric acid levels in the blood.

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Hypouricemia Causes

May be due to low renal threshold or decreased uric acid formation.

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Xanthine Oxidase Deficiency

Deficiency leads to excess excretion of hypoxanthine and xanthine in urine.

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β-Alanine

Uracil and cytosine catabolism end product.

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β-Amino Isobutyric Acid

Thymine catabolism end product.

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β-Amino Isobutyric Aciduria

Excretion of large amounts of β-aminoisobutyric in urine.

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Hereditary Orotic Aciduria Type I

Defective orotate phosphoribosyltransferase and orotidine 5'-phosphate decarboxylase.

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Hereditary Orotic Aciduria Type II

Defective OMP decarboxylase.

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Hereditary Orotic Aciduria Symptoms

Retarded growth, severe anemia, megaloblastic bone marrow.

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What are the main Purines?

Purines include?

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What are the main Pyrimidines?

Pyrimidines include?

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Dietary source of Purines and Pyrimidines

Source in liver, meats, and legumes

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What is Gout?

A metabolic disorder linked to high levels of uric acid

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Catabolism location of pyrimidines

Where does catabolism of Pyrimidines occur?

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Study Notes

Purines and Pyrimidines

  • Purines and pyrimidines are heterocyclic aromatic compounds that form the important components of nucleotides along with sugar and phosphate groups.
  • Purines include adenine and guanine, while pyrimidines include thymine, uracil, and cytosine.

Nucleotide Structure

  • A nucleoside is formed from the linkage of a sugar with a nitrogen-containing base.

Biomedical Importance

  • DNA and RNA are nucleic acids composed of purines and pyrimidines.
  • ATP and GTP are energy carriers.
  • NADH and FADH are co-enzymes.
  • S-Adenosyl-methionine is a donor for methyl groups.
  • UDP-glucuronic acids are donors for conjugation (e.g., bilirubin glucuronide).
  • cAMP and cGMP are involved in hormone signaling.
  • Anticancer drugs (e.g., MTX, 5-Flu, 6-TG, 6-MG) and antiviral drugs (e.g., Aciclovir, Lamivudine) are developed based on purine and pyrimidine metabolism.
  • Allopurinol is used to treat gout disease.

Dietary Non-Essentiality

  • Human tissues can synthesize purines and pyrimidines, making them dietarily non-essential.
  • Ingested nucleic acids and nucleotides are degraded in the intestinal tract to mononucleotides, which may be absorbed or converted to purine and pyrimidine bases.
  • Dietary sources of purines and pyrimidines include liver, meat, legumes, cereals, and vegetables, as well as tea, coffee, cocoa, and cola.

Purine and Pyrimidine Metabolism

  • Digestion and absorption: pancreatic juice contains nucleases that hydrolyze nucleic acids into mononucleotides, which are then broken down by nucleotidases and intestinal phosphatases.
  • Unabsorbed purines are converted to uric acid by intestinal bacteria, which may be absorbed and excreted in urine.

Catabolism of Purine

  • Site: liver (intracytoplasmic)
  • Hypoxanthine is oxidized into xanthine, and further into uric acid by xanthine oxidase.
  • Uric acid diffuses to the blood to be excreted by the kidney.
  • Uric acid is the end product of purine base catabolism.

Gout

  • Gout is a metabolic disorder related to excess production and deposition of uric acid crystals (hyperuricemia).
  • Hyperuricemia is characterized by recurrent attacks of acute inflammatory arthritis.
  • Normal blood uric acid levels are 3-7 mg/dl (males) and 2-6 mg/dl (females).
  • It is excreted in urine (0.7 gm/day), with decreased excretion in cases of purine-free diet.

Lesch-Nyhan Syndrome

  • Lesch-Nyhan syndrome is an X-linked recessive disorder caused by defects in the activity of the purine nucleotide salvage enzyme, hypoxanthine-guanine phosphoribosyl transferase.
  • Causes: deficiency of the enzyme HGPRT, produced by mutations in the HPRT gene.
  • Symptoms: severe gout, kidney problems, poor muscle control, and moderate mental retardation.

Hypouricemia

  • It may be renal (due to low renal threshold) or metabolic (due to decreased uric acid formation).
  • Causes of metabolic hypouricemia:
    • Deficiency of adenosine deaminase (adenosinuria)
    • Deficiency of xanthine oxidase (hypoxanthine and xanthine are excreted in excess in urine)
    • Deficiency of purine nucleosidase (accumulation of guanosine and inosine leading to guanosinuria)
    • Deficiency of folic acid (decreased de novo synthesis of purines)

Catabolism of Pyrimidines

  • This occurs mainly in the liver.
  • β-Alanine is the major end product of both uracil and cytosine.
  • β-Amino isobutyric is the major end product of thymine.

Disorders of Pyrimidine Metabolism

  • β-Amino isobutyric aciduria: characterized by excretion of large amounts of β-aminoisobutyric in urine, caused by defective deamination of β-aminoisobutyric acid.
  • Hereditary orotic aciduria:
    • Type I: caused by defective orotate phosphoribosyl transferase and orotidine 5'-phosphate decarboxylase, leading to retarded growth, severe anemia, and megaloblastic bone marrow.
    • Type II: caused by defective OMP decarboxylase, similar to Type I.

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Description

This quiz covers the components of nucleotides, including purines and pyrimidines, and their role in DNA and RNA. Test your knowledge of adenine, guanine, thymine, uracil, and cytosine.

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