Question

glucose-6-phosphate dehydrogenase (G6PD) deficiency



I'm researching G6PD deficiency and i'm stuck at this. Fava beans contains the alkaloïds vicine and covicine. What do these 2 exactly trigger in the pentosephosphatecascade on molecular level? I know that G6PD converts NADP+ to NADPH which is then converted back by glutathione reductase. glutathone reductase then reduces glutathione. The reduced glutathione can mop away free radicals, which prevents oxidative stress.

Answer 1

So G6PD deficiency prevents glutatione to become reduced and oxidative stress will occur in red blood cells. Due to following hemolysis bilirubin goes in the bloodstream causing jaundice.

BUT, why do compounds like vicine, covicine, naftalene, etc triggers this mass hemolysis?

Answer 2

This is the molecular pathology of G6PD deficiency.

Answer 3

Do vicine, covicine or naphthalene have something to do with free radicals?

Answer 4

Vicine and convicine are pyrimidine glucoside that are abundant in fava beans and make up 6.7 g/100 g of dry weight of fava beans (67). These substances are metabolized by B-glucosidase in body to divicine and isoramil that are unstable aglycons, and are oxidized rapidly and form hy- drogen peroxide and superoxide anion. These me- tabolites oxidize reduced glutathione in normal and G6PD-deficient red blood cells


http://journals.tums.ac.ir/upload_files/pdf/_/12366.pdf

Answer 5

wow thanks, that article helped alot!