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Objective  Pelizaeus–Merzbacher disease ( PMD ) is a progressive and lethal leukodystrophy caused by mutations affecting the proteolipid protein (  PLP 1 ) gene. The most common cause of  PMD  is a duplication of   PLP 1  and at present there is no curative therapy available.    Methods  By using transgenic mice carrying additional copies of  Plp1 , we investigated whether curcumin diet ameliorates  PMD  symptoms. The diet of  Plp1  transgenic mice was supplemented with curcumin for 10 consecutive weeks followed by phenotypical, histological and immunohistochemical analyses of the central nervous system.  Plp1  transgenic and wild‐type mice fed with normal chow served as controls.    Results  Curcumin improved the motor phenotype performance of  Plp1  transgenic mice by 50% toward wild‐type level and preserved myelinated axons by 35% when compared to  Plp1  transgenic controls. Furthermore, curcumin reduced astrocytosis, microgliosis and lymphocyte infiltration in  Plp1  transgenic mice. Curcumin diet did not affect the pathologically increased  Plp1   mRNA  abundance. However, high glutathione levels indicating an oxidative misbalance in the white matter of  Plp1  transgenic mice were restored by curcumin treatment.    Interpretation  Curcumin may potentially serve as an antioxidant therapy of  PMD  caused by   PLP 1  gene duplication.

Curcumin therapy in a Plp1 transgenic mouse model of Pelizaeus‐Merzbacher disease