0.10 Carbohydrates iv

1. A 21-year old male joins the military. He is required to do exercise during basic training. He experiences pain, cramps and fatigue after 20 minutes of strenuous physical activity and his urine turns burgundy-colored. Lab results showed after exercise: high serum creatine levels and myoglobinuria. An ischemic forearm exercise test showed: low lactate and high ammonia levels in blood. A muscle biopsy reveals:

Glycogen 1.85 mmol/min/g ( Normal values: 0.1 - 1.5 mmol/min/g)

Phosphorylase A 0.09 mmol/min/g ( Normal values: 12 mmol/min/g)

What condition is this patient suffering from?

1. McArdles' disease type V
2. Andersons' disease type IV
3. von Gierke's disease type I
4. Hers' disease type VI
5. Cori's disease type III

2. Which of the following hepatic enzymes connects glycogenolysis to the last step of gluconeogenesis?

1. Glycogenin
2. Branching enzyme
3. Phosphoglucomutase
4. Glucose-6-phosphatase
5. Glycogen phosphorylase

3. Which of the following proteins is directly involved in glycogenolysis in the liver and muscle cells?

1. Glycogenin
2. Phosphoglucomutase
3. Branching enzyme
4. Glycogen synthase
5. Insulin

4. Which of the following statements about fibers is INCORRECT ?

1. Soluble fibers are fermented by normal intestinal bacteria.
2. Soluble fibers slow the absorption of glucose into circulation.
3. Soluble fibers can prevent heart disease by reducing cholesterol in circulation.
4. Insoluble fibers increase the regularity of bowel movements and prevent constipation.
5. The products of insoluble fibers are gases, short fatty acids, lactate and vitamin K.

5. What is the main purpose of glucuronidation reactions?

1. Stimulate abnormal cell proliferation in the tissues.
2. Activate other enzymes to release non-polar molecules.
3. Get rid of drugs faster from the body without a therapeutic effect.
4. Add two glucose molecules to non-polar molecules to make them polar.
5. Clear insoluble molecules by excreting them via the urine and feces.

6. Which of the following enzymes is responsible for the development of cataracts in galactosemic patients?

1. Galactose-1-phosphate uridyltransferase
2. UDP-galactose-4-epimerase
3. Sorbitol dehydrogenase
4. Galactokinase
5. Aldose reductase

7. Which of the following enzymes actively breaks down complex carbohydrates in both the mouth and the lumen of the intestines?

1. α-amylase
2. Sucrase
3. Isomaltase
4. ß-glucoamylase
5. ß-glycosidase

8. Which of the following is the best screening test to detect GLUT1 mutations?

1. CSF glucose levels
2. Serum glucose levels
3. SLC2A1 genetic testing
4. CSF per Blood glucose ratios
5. F 18 positron emission tomography

9. Which of the following proteins is found in high concentration in colostrum?

1. α-lactalbumin
2. Immunoglobulin A
3. Lysozyme
4. Insulin
5. Casein

10. A 7-month old baby is being introduced to solid foods. Mother notices her baby gets fussy, and suffers from chronic bloating and diarrhea every time he eats bananas, apples or pears. A stool reducing substances test is positive. Which of the following proteins might be defective in this patient?

1. Sucrase
2. α-amylase
3. ß-glucoamylase
4. GLUT5
5. ß-glycosidase

11. Which of the following enzymes can only break disaccharides inside the lumen of the intestines?