Review Questions

Select the ONE best answer-

1. As part of a study to quantify contributors of stress to hyperglycemia and ketosis in diabetes, normal hepatocytes and adipocytes in tissue culture were treated with Cortisol and analyzed by Northern blotting using a gene-specific probe. The results of one experiment are shown below.

Hepatocytes Cortisol (nM) 0 100 500 1000

Adipocytes Cortisol (nM)

0 100 500 1000

Hepatocytes Cortisol (nM) 0 100 500 1000

Adipocytes Cortisol (nM)

0 100 500 1000

The 32P-probe used in this experiment most likely binds to a mRNA encoding

A. phosphoenolpyruvate carboxykinase

B. lipoprotein lipase

C. glucokinase

D. hormone-sensitive lipase

E. acetyl-CoA carboxylase

2. A child is diagnosed with a congenital deficiency of medium-chain acyl-CoA dehydrogenase activity. Which of the following signs or symptoms would most likely occur upon fasting in this child?

A. Hypolacticacidemia

B. Ketoacidosis

C. Hyperglycemia

D. Dicarboxylic acidosis

E. Hyperchylomicronemia

3. A 14-year-old boy has been experiencing progressive onset of muscle fatigue and cramping. His physician finds no evidence for hypoglycemia, and fatty acids are released appropriately in response to a glucagon challenge. A muscle biopsy reveals unusual lipid-filled vacuoles in the cytoplasm of his myocytes. On analysis the vacuoles are found to contain triglyceride. The most likely cause of these symptoms is a deficiency of

A. muscle phosphorylase

B. hepatic phosphorylase

C. debranching enzyme

D. muscle carnitine

E. apoB-100 receptor

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10. A 40-year-old woman with a history of bleeding and pancytopenia now presents with leg pain. She describes a deep, dull pain of increasing severity that required pain medication. Computed tomography examination reveals erosion and thinning of the femoral head. A bone marrow biopsy is performed to confirm a diagnosis of Gaucher disease. What material would be found abnormally accumulating in the lysosomes of her cells?

A. Mucopolysaccharide

B. Ganglioside

C. Ceramide

D. Cere bro side

E. Sulfatide

11. An underweight 4-year-old boy presents semi-comatose in the emergency room at 10 a.m. Plasma glucose, urea, and glutamine are abnormally low; acetoacetate is elevated; and lactate is normal. He is admitted to the ICU, where an increase in blood glucose was achieved by controlled infusion of glucagon or alanine. Which metabolic pathway is most likely deficient in this child?

A. Hepatic gluconeogenesis

B. Skeletal muscle glycogenosis

C. Adipose tissue lipolysis

D. Skeletal muscle proteolysis

E. Hepatic glycogenosis

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