JOY CZS Pyridoxine has multiple mechanisms of action in responsive PH1 patients. Pyridoxine works via PLP as a chemical chaperone and a prosthetic group. Pyridoxine increases the expression, activity and peroxisomal targeting of AGT-170. Pyridoxine enhances the functionality of AGT-170, AGT-152 and AGT-244 in CHO cells
Nicotinamide replenishes cellular energy as it is a precursor of NAD and NADP. Secondly, it prevents overactivation of PARP-1 by negative feedback, thus preventing cell senescence
In the blood, it attaches itself to plasma proteins. Tissues absorb vitamin B12 by specific B12 binding proteins, transcobalamin I and II, allowing it to enter the cells. Most of the vitamin is stored in the liver
The mechanism by which folic acid prevents structural anomalies in the fetus is not known, but may involve the regulation of homocysteine metabolism. Several investigators have suggested that folic acid supplementation may have additional benefits on pregnancy outcome.
An exogenous source of folate is required for nucleoprotein synthesis and the maintenance of normal erythropoiesis. Folic acid, whether given by mouth or parenterally, stimulates the production of red blood cells, white blood cells, and platelets in persons suffering from certain megaloblastic anemias.
Chromium helps to maintain normal glucose metabolism and peripheral nerve function. Chromium increases insulin binding to cells, increases insulin receptor density and activates insulin receptor kinase leading to enhanced insulin sensitivity
Zinc can treat acute and chronic diarrhea by inhibiting three out of the four main intracellular pathways of intestinal ion secretion, including cyclic adenosine monophosphate (cAMP), calcium, and nitric oxide
Selenium serves as a cofactor for glutathione peroxidase and helps minimize oxidative damage through cellular metabolism. [6] Selenium, in combination with vitamin E, protects cell membranes and organelles from peroxidative destruction.