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Biochemistry · Carbohydrate metabolism: fructose
Sample itemAn infant develops vomiting, jaundice and hypoglycaemia after fruit juice is introduced. Accumulation of fructose-1-phosphate points to deficiency of which enzyme?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Aldolase B
Hereditary fructose intolerance results from aldolase B deficiency. Trapped fructose-1-phosphate depletes phosphate and impairs gluconeogenesis and glycogenolysis.
Option by option
A. Aldolase B (correct)
Correct. Aldolase B cleaves fructose-1-phosphate.
B. Phosphoglucomutase
Phosphoglucomutase interconverts glucose-1-phosphate and glucose-6-phosphate.
C. Fructokinase
Fructokinase deficiency causes benign essential fructosuria.
D. Galactokinase
Galactokinase deficiency causes galactitol-related cataracts.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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