Biochem Mehlman QUESTION AND ANSWER REVISION TEST

High plasma glucose levels (i.e., in diabetes mellitus) = high intracellular sorbitol levels. What intracellular enzyme that converts glucose to sorbitol? aldose reductase Sorbitol can _________________________________________ cause osmotic injury to cells because it leads to greater intracellular retention of water relative to glucose, thereby causing cellular swelling how to cataracts or neuropathy develop in diabetes due to osmotic injury to cells nephropathy, retinopathy, and atherosclerosis seen in diabetes are due to: non-enzymatic glycosylation of endothelial cells by glucose, which leads to direct cellular damage. What converts Galactose → galactitol aldose reductase Galactose-1-phosphate uridylyltransferase (GALT) deficiency causes classic galactosemia Sx: presentation is severe, with jaundice, hepatosplenomegaly, cataracts, and mental impairment. required cofactor for conversion of glucose to sorbitol NADPH Galactokinase deficiency -infantile cataracts and galactose in the urine -an infant who recently started breastfeeding who now has problems seeing or who has leukocoria. -“a reducing sugar” was detected in the urine. classic galactosemia -present severely.