Nusinersen mitigates neuroinflammation in severe spinal muscular atrophy patients.

ABSTRACT:

Background

Neuroinflammation contributes to the onset and progression of neurodegenerative diseases, but has not been specifically investigated in patients affected by severe and milder forms of spinal muscular atrophy (SMA).

Methods

In this two-center retrospective study, we investigated signatures of neuroinflammation in forty-eight pediatric male and female SMA1 (n = 18), male and female SMA2 (n = 19), and female SMA3 (n = 11) patients, as well as in a limited number of male and female non-neurological control subjects (n = 4). We employed a Bio-Plex multiplex system based on xMAP technology and performed targeted quantitative analysis of a wide range of pro- and anti-inflammatory cytokines (chemokines, interferons, interleukins, lymphokines and tumor necrosis factors) an

SUBMITTER: Nuzzo T 

PROVIDER: S-EPMC9932014 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature