Which statement best explains the structural basis for the higher energy content of glucose compared to other hexoses?
AGlucose has more hydroxyl groups
BGlucose is more stable and requires less energy for phosphorylation
CAll hexoses have identical energy content; ATP yield depends on metabolic pathway efficiency
DGlucose has a unique aldehyde group arrangement
Correct Answer:
C. All hexoses have identical energy content; ATP yield depends on metabolic pathway efficiency
EXPLANATION
All hexoses yield approximately the same amount of ATP (~32-38 ATP) through complete oxidation. The ATP yield depends on the metabolic pathways utilized, not intrinsic energy differences.
In hereditary fructose intolerance (HFI), which enzyme deficiency causes accumulation of fructose-1-phosphate?
AFructokinase
BAldolase B
CFructose-1,6-bisphosphatase
DFructose-1-phosphatase
Correct Answer:
B. Aldolase B
EXPLANATION
HFI results from aldolase B deficiency, preventing cleavage of fructose-1-phosphate into DHAP and glyceraldehyde, leading to accumulation and hepatotoxicity.
A newborn presents with hepatomegaly, lactic acidosis, and hypoglycemia. Enzyme analysis shows deficiency of glucose-6-phosphatase. Which disease is this?
AMcArdle disease
BPompe disease
CVon Gierke disease
DCori disease
Correct Answer:
C. Von Gierke disease
EXPLANATION
Von Gierke disease (GSD Type I) results from glucose-6-phosphatase deficiency, preventing final step of both gluconeogenesis and glycogenolysis, causing severe hypoglycemia.
Which of the following is NOT a function of glycogen in liver?
AMaintaining blood glucose levels between meals
BProviding structural support to hepatocytes
CActing as glucose buffer during fasting
DSupplying glucose-6-phosphate to extrahepatic tissues
Correct Answer:
D. Supplying glucose-6-phosphate to extrahepatic tissues
EXPLANATION
Liver glycogen maintains blood glucose but cannot directly supply glucose-6-phosphate to other tissues as glucose-6-phosphate cannot cross cell membranes.
During high-intensity exercise, which carbohydrate provides immediate energy despite low concentration in blood?
ABlood glucose from liver glycogenolysis
BMuscle glycogen
CCirculating fructose
DDietary disaccharides
Correct Answer:
B. Muscle glycogen
EXPLANATION
Muscle glycogen is the primary energy source during high-intensity exercise because muscle lacks glucose-6-phosphatase and retains glucose-6-phosphate for glycolysis.
Correct Answer:
B. Galactose-1-phosphate uridyltransferase deficiency
EXPLANATION
Classical galactosemia results from galactose-1-phosphate uridyltransferase (GALT) deficiency, causing accumulation of galactose-1-phosphate and galactosylated proteins.