Understanding Metabolic Processes: From Krebs Cycle to Glycogen Storage Diseases
The Krebs Cycle and Cellular Respiration
Krebs Cycle Basics
1. ATP Production: The Krebs cycle generates 1-12 moles of ATP per cycle.
2. Oxidative Decarboxylation: It’s a multi-stage process of oxidative decarboxylation.
3. Key Molecules Involved: The reactions in the Krebs cycle involve NAD+, FAD, and lipolic acid.
4. Intermediate Molecules:
- Succinyl CoA
- NADH+, H+
- Oxaloacetate, acetyl-CoA, and water
- Citric acid
5. Location: The Krebs cycle takes place in the mitochondrial matrix and the inner membrane of the mitochondria.
6. Acetyl-CoA Integration: The rest of Acetyl-CoA is continuously incorporated into the cycle.
7. NAD+ Regeneration: NAD+ is regenerated during the cycle.
8. Connections to Other Pathways: The Krebs cycle is linked to the oxidative deamination of glutamic acid.
Metabolic Processes and Disorders
9. Malic Acid Oxidation: Oxidation of malic acid is a key step in the cycle.
10. NADH Dehydrogenase: NADH dehydrogenase is an important enzyme in the electron transport chain.
11. Proton Movement: Protons cross through proton channels into the mitochondrial matrix, driving ATP synthesis.
12. ATP Synthase: ATP synthase activity is crucial for ATP production.
13. Glycogen Metabolism:
- UDP glucose is involved in glycogen synthesis.
- Glycogen phosphorylase breaks down glycogen.
14. Galactosemia: Galactosemia is caused by aldose reductase deficiency.
Glycogen Storage Diseases
Glycogen Storage Disease Type II (Pompe Disease)
20. True or False:
| a. Deficiency of acid maltase | T |
| b. Normal blood sugar levels and glycogen structure | F |
| c. Is a lysosomal storage disease | T |
Other Metabolic Disorders
21. Aldolase B Deficiency: Absence of aldolase B can disrupt glycolysis.
22. Renal Threshold for Glucose: Health issues can arise when the renal threshold for glucose is exceeded, such as during pregnancy or in neonates.
23. Sarcoplasmic Calcium: An increase in sarcoplasmic calcium levels can impact muscle function.
24. Glucose 6-Phosphate Dehydrogenase Deficiency: This deficiency can lead to hemolytic anemia.
25. Lactase: Lactase is an enzyme that breaks down lactose.
McArdle Syndrome
27. True or False:
| a. Temporary weakness and cramping of skeletal muscle after exercise | T |
| b. Skeletal muscle glycogen phosphorylase deficiency | T |
| c. Progressive renal disease | F |