Entrance Exams
Govt. Exams
HFI results from aldolase B deficiency, preventing cleavage of fructose-1-phosphate into DHAP and glyceraldehyde, leading to accumulation and hepatotoxicity.
Von Gierke disease (GSD Type I) results from glucose-6-phosphatase deficiency, preventing final step of both gluconeogenesis and glycogenolysis, causing severe hypoglycemia.
Liver glycogen maintains blood glucose but cannot directly supply glucose-6-phosphate to other tissues as glucose-6-phosphate cannot cross cell membranes.
Muscle glycogen is the primary energy source during high-intensity exercise because muscle lacks glucose-6-phosphatase and retains glucose-6-phosphate for glycolysis.
Branching enzyme (amylo-1,6-transglucosidase) transfers segments of α-1,4-linked glucose chains to create α-1,6 branch points in glycogen.
Classical galactosemia results from galactose-1-phosphate uridyltransferase (GALT) deficiency, causing accumulation of galactose-1-phosphate and galactosylated proteins.
Phosphoglucose isomerase catalyzes the interconversion of glucose-6-phosphate and fructose-6-phosphate in glycolysis.
Sucrose is composed of glucose and fructose linked by α-1,2-glycosidic bond. Maltose has α-1,4 bond between two glucose molecules.
Fructose is a ketohexose (6-carbon ketone sugar), while glucose, galactose are aldohexoses, and ribose is a pentose.
The Warburg effect describes the metabolic shift in cancer cells toward anaerobic glycolysis, producing lactate even in the presence of oxygen, resulting in high glucose consumption.