Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water.

ABSTRACT: CLN3 mutations cause the fatal neurodegenerative disorder, CLN3 Batten disease. The Cln3^-/- mouse model displays characteristic features of the human disease including motor deficits. When mice received acidified drinking water (pH 2.5-2.9) instead of normal tap water (pH 8.4) for several generations, the motor skills of Cln3^-/- mice normalized to control levels, indicating a disease-modifying effect of acidified water. Here we investigated if acidified water administered from postnatal day 21 has therapeutic benefits in Cln3^-/- mice. Indeed, acidified water temporarily attenuated the motor deficits, had beneficial effects on behavioral parameters and prevented microglial activation in the brain of Cln3^-/- mice. Interestingly, in control mice, ac

SUBMITTER: Johnson TB 

PROVIDER: S-EPMC6802212 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature