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12- Biochemistry 12 (2 Hours & 19 minutes)

[vdo id=’6fd562a1506c474db3e74f870048c051′]

 

   Content of this Session
  • Glycolysis
  • Galactose metabolism
  • Galactokinase deficiency
  • Classic galactosemia
  • Sorbitol
  • Fructose metabolism
  • Essential fructosuria
  • Hereditary fructose intolerance

 

 

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[h] Biochemistry Flashcards

[i] Master this session in just 5 minutes.

[q] Glucose entering the cell is trapped by phosphorylation using ATP. ……… is widely distributed in tissues, whereas ………. is found only in hepatocytes and pancreatic β-islet cells.

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[q] ……. is the rate-limiting enzyme and main control point in glycolysis. In this reaction, fructose 6-phosphate is phosphorylated to fructose 1,6-bisphosphate using ATP.

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[q] Insulin activates …….., which converts a tiny amount of fructose 6-phosphate to fructose 2,6-bisphosphate (F2,6-BP). F2,6-BP activates PFK-1.

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[q] ……….. is the last enzyme in aerobic glycolysis which catalyzes a substrate-level phosphorylation of ADP using the high-energy substrate phosphoenolpyruvate (PEP).

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[q] ………. is used only in anaerobic glycolysis. It reoxidizes NADH to NAD, replenishing the oxidized coenzyme for glyceraldehyde 3-phosphate dehydrogenase by reducing pyruvate to lactate and oxidizing NADH to NAD.

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[q] ………. yields 2 ATP/glucose by substrate-level phosphorylation. But ………. yields these 2 ATP/glucose plus 2 NADH/glucose that can be utilized for ATP production in the mitochondria.

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[q] In red blood cells, ……… represents the only pathway for ATP production, yielding a net 2 ATP/glucose.

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[q] Erythrocytes have bisphosphoglycerate mutase, which produces 2,3-bisphosphoglycerate (BPG) from 1,3-BPG in glycolysis. This reaction bypasses an ATP-generating step of glycolysis, causing ……..?

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[q] 2,3-BPG binds to the β-chains of hemoglobin A (HbA) and …… its affinity for oxygen.

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[q] Excessive erythrocyte destruction in pyruvate kinase deficiency by the spleen causes splenomegaly due to ……..?

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[q] In patients with ………. deficiency, glycolysis is inhibited in strenuously exercising muscle as muscle cells cannot regenerate NAD. Consequently, high-intensity physical activity leads to muscle breakdown, pain, and fatigue as insufficient amounts of energy are being produced in the exercising muscle.

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[q] What is the most likely diagnosis?

7 years old child presenting with galactosemia, galactosuria, and infantile cataracts?

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[Qq]

[q] What is the most likely diagnosis?

Newborn child presenting with failure to thrive, jaundice, hepatomegaly, and renal dysfunction (hyperchloremic metabolic acidosis, aminoaciduria), infantile cataracts, intellectual disability after beginning of breastfeeding?

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[q] In the polyol pathway, aldose reductase converts glucose into ………, which is slowly metabolized into ………. by sorbitol dehydrogenase.

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[q] What is the most likely diagnosis?

21 years old patient presenting with fructosuria + the presence of reducing sugar in the urine + urine dipstick, however, is negative without any other significant symptoms?

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[q] What is the most likely diagnosis?

6 month old child presenting with Hypoglycemia, lethargy, vomiting, jaundice after after beginning to introduce fruits to the diet + Urine dipstick is ⊝ although reducing sugar are detected in the urine?

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[q] Glucose-6-phosphate, mannose-6-phosphate, galactose-6-phosphate and glucose-1-phosphate must be metabolized via the rate-limiting PFK-1 step, which slows down their metabolism. But ……….., however, bypasses this step and therefore has the highest rate of metabolism.

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[x][restart]

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