Heme Synthesis Regulation

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Questions and Answers

What is the significance of positive cooperativity in hemoglobin?

  • It increases the affinity for O2 in adjacent subunits (correct)
  • It decreases the affinity for O2 in adjacent subunits
  • It allows hemoglobin to deliver less O2 to the tissues
  • It leads to a linear shape of the oxygen-hemoglobin dissociation curve

Which of the following is NOT a positive effector that increases oxygen binding to hemoglobin?

  • Low H+ (high alkaline pH)
  • High concentration of 2,3-bisphosphoglycerate (BPG) (correct)
  • Low concentration of a special metabolite of erythrocytes
  • Low CO2

Where does heme synthesis mainly occur?

  • Ribosomes
  • Mitochondria and cytosol (correct)
  • Golgi apparatus
  • Endoplasmic reticulum

What is the negative effect of negative effectors on hemoglobin?

<p>Decrease binding of oxygen and facilitate its delivery to tissue (D)</p> Signup and view all the answers

What is the role of heme in hemoglobin?

<p>Participates in the structure of hemoglobin (D)</p> Signup and view all the answers

Which of the following is true about 2,3-bisphosphoglycerate (BPG)?

<p>It's a positive effector that decreases oxygen binding to hemoglobin (C)</p> Signup and view all the answers

What is the location of heme synthesis for hemoglobin production?

<p>Red blood cell progenitor cells (B)</p> Signup and view all the answers

Which enzyme deficiency is associated with Acute Intermittent Porphyria?

<p>Porphobilinogen deaminase (D)</p> Signup and view all the answers

What are the typical symptoms of Porphyria cutanea tarda?

<p>Photosensitivity and dark red/brown colored urine (A)</p> Signup and view all the answers

Which pathway intermediate accumulation causes neurological symptoms in Porphyrias?

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

Which type of Porphyria does NOT exhibit photosensitivity?

<p>Acute intermittent porphyria (A)</p> Signup and view all the answers

What is the key pathway intermediate that leads to the characteristic symptoms of Porphyrias?

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

Which enzyme deficiency characterizes Porphyria cutanea tarda?

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

Which of the following drugs can worsen the symptoms of Porphyrias by stimulating the heme synthesis pathway?

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

Where does the degradation of abnormal red blood cells primarily occur?

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

Which enzyme is the rate-limiting enzyme in heme synthesis?

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

What inhibits ALA synthase in the liver when there is an excess of heme?

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

Lead poisoning primarily affects which enzymes in heme synthesis?

<p>ALA synthase &amp; Ferrochelatase (C)</p> Signup and view all the answers

Which deficiency leads to the inhibition of ALA synthase?

<p>Vitamin B6 deficiency (C)</p> Signup and view all the answers

What role does vitamin B6 play in heme synthesis?

<p>Cofactor for ALA synthase (C)</p> Signup and view all the answers

How does lead poisoning differentiate itself from porphyrias?

<p>By inducing ALA dehydratase deficiency without a PBG increase (B)</p> Signup and view all the answers

Which enzyme introduces Fe2+ into the heme ring in the final step of heme synthesis?

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

What is the final product of heme degradation in macrophages?

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

How is iron released from hemoglobin in macrophages stored?

<p>Bound to ferritin (B)</p> Signup and view all the answers

What enzyme reduces biliverdin to bilirubin in the cytosol?

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

In what form is bilirubin transported in the blood?

<p>Bound to serum albumin (D)</p> Signup and view all the answers

Which of the following can be excreted in urine?

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

What is the fate of most urobilinogen produced in the gastrointestinal tract?

<p>Excreted in feces as stercobilin (A)</p> Signup and view all the answers

What can occur if there is an accumulation of unconjugated bilirubin in the basal ganglia of the brain?

<p>Death or mental retardation (A)</p> Signup and view all the answers

How is bilirubin converted to urobilinogen in the gastrointestinal tract?

<p>Reduction by intestinal bacteria (A)</p> Signup and view all the answers

What is the correct composition of globin chains in a hemoglobin molecule?

<p>2 α and 2 β subunits (C)</p> Signup and view all the answers

How many pyrolle rings are present in the heme structure?

<p>4 (A)</p> Signup and view all the answers

In what state does hemoglobin have a higher affinity for O2?

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

How many oxygen molecules can each Hb molecule bind to?

<p>4 (C)</p> Signup and view all the answers

Which amino acid binds to the 5th coordination bond of iron in the heme group?

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

In which state does deoxygenated hemoglobin exist?

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

What is the oxidation state of iron in the heme moieties?

<p>Ferrous (Fe2+) (C)</p> Signup and view all the answers

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