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Continuous leaking of mitochondrial CoA through the PTP leads to the inability of the cell to build a sufficient mitochondrialcytoplasmic CoA gradient, also causing insufficient release of mitochondrial CoA as a response to cell stress and injury. Decreased palmitoylation capacity and decreased endocytosis and membrane remodelling are implicated in proven pathophysiological mechanisms in INAD and DMD. The described mechanism in INAD and DMD, may be considered a common mechanism of repair in case of cell stress and injury. Beside their