Question

People with galactosemia display central nervous system abnormalities even if galactose 9 eliminated from the diet. The precise reason is not know. Suggest an explanationanner

Answer 1

3. Galactosemia ocurs due to the deficiency of  galactose-1-phosphate uridylyltranserase (GALT) enzyme, which leads to accumulation of galactose-1- phosphate in brain, liver and blood cells. Inspite of diet, it affects the CNS leads to neurological, cognitive and behavioural disorders. Although the galactose foods are avoided in diet, the accumulation of galactose-1- phosphate in brain increases the CNS problems.

5. The important regulatory step in glycolysis is phosphofructokinase reaction. PFK is regulated by the high energy bonds of adenosine nucleotides of the cell. Pyruvate kinase reaction is very important in controlling the flux of fructose in the liver, which also enters glycolysis after the Phosphofructokinase reaction.

6. Hexokinase D plays an important role in the metabolism of glucose . Hence, following a carbohydrate meal definitely the blood glucose level will be higher .

11. Usually kinases are enzymes that catalyzes the transfer/ donating high energy phosphate groups to the specific substrates by the process of phosphorylation. Succinyl CoA synthetase (SCS) catalyzes the substrate level phosphorylation in the krebs cycle , also it has histidine phosphorylation site , hence SCS is considered as kinases enzymes.