Acidified drinking water attenuates motor deficits and brain pathology in a mouse model of a childhood neurodegenerative disorder.

ABSTRACT: We recently demonstrated that HCl-acidified drinking water, which is widely used in laboratory animal facilities, had some beneficial effects in the Cln3^-/- mouse model of juvenile Batten disease, a neurodegenerative lysosomal storage disorder¹. Here we tested if acidified drinking water has therapeutic effects in Cln1^R151X nonsense mutant mice, a model of the infantile form of Batten disease. In Cln1^R151X mice, acidified drinking water received from weaning prevented the impairment in pole climbing ability measured at 3 and 6 months of age. Histopathological analysis of the brain at 6 months showed that acidified drinking water decreased the amount of lysosomal storage material, reduced astrocytosis in the striatum and somatosensory barrelfield

SUBMITTER: Kovacs AD 

PROVIDER: S-EPMC9151921 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature