Thank you very much! 9:00 I wanted to add that there are two new FDA-approved drugs called "tetrahydrobiopterin" (cofactor for phenylalanine hydroxylase) and "pegvaliase" that can be used as an addon to the phenylalanine-restricted diet. 11:00 Afaik at the age of 5 an alternate pathway for the galactose metabolism will be available, hence it would be okay to consume galactose. 19:00 "Betaine is used to reduce concentrations of homocysteine by promoting the conversion of homocysteine back to methionine, i.e., increasing flux through the re-methylation pathway independent of folate derivatives (which is mainly active in the liver and in the kidneys). The re-formed methionine is then gradually removed by incorporation into body protein. The methionine that is not converted into protein is converted to S-adenosyl-methionine which goes on to form homocysteine again. Betaine is, therefore, only effective if the quantity of methionine to be removed is small. Hence treatment includes both betaine and a diet low in methionine. In classical homocystinuria (CBS, or cystathione beta synthase deficiency), the plasma methionine level usually increases above the normal range of 30 micromoles/L and the concentrations should be monitored as potentially toxic levels (more than 400 micromoles/L) may be reached." Wiki :-)
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