Anle138b modulates α-synuclein oligomerization and prevents motor decline and neurodegeneration in a mouse model of multiple system atrophy.

ABSTRACT:

Background

MSA is a fatal neurodegenerative disease characterized by autonomic failure and severe motor impairment. Its main pathological hallmark is the accumulation of α-synuclein in oligodendrocytes, leading to glial and neuronal dysfunction and neurodegeneration. These features are recapitulated in the PLP-hαSyn mouse model expressing human α-synuclein in oligodendrocytes. At present, there is no effective disease-modifying therapy. Previous experiments have shown that the aggregation inhibitor, anle138b, reduces neurodegeneration and behavioral deficits in mouse models of other proteinopathies.

Objectives

To test the therapeutic potential of anle138b in a mouse model of MSA.

Methods

Two-month-old PLP-hαSyn mice were fed over a period of 4 months with pellets cont

SUBMITTER: Heras-Garvin A 

PROVIDER: S-EPMC6492169 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature