P2X7 antagonism using Brilliant Blue G reduces body weight loss and prolongs survival in female SOD1^G93A amyotrophic lateral sclerosis mice.

ABSTRACT:

Background

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease characterised by the accumulation of aggregated proteins, microglia activation and motor neuron loss. The mechanisms underlying neurodegeneration and disease progression in ALS are unknown, but the ATP-gated P2X7 receptor channel is implicated in this disease. Therefore, the current study aimed to examine P2X7 in the context of neurodegeneration, and investigate whether the P2X7 antagonist, Brilliant Blue G (BBG), could alter disease progression in a murine model of ALS.

Methods

Human SOD1^G93A transgenic mice, which normally develop ALS, were injected with BBG or saline, three times per week, from pre-onset of clinical disease (62-64 days of age) until end-stage. Duri

SUBMITTER: Bartlett R 

PROVIDER: S-EPMC5335685 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature