Gamma-synuclein pathology in amyotrophic lateral sclerosis.

ABSTRACT:

Objective

The prominent histopathological feature of the amyotrophic lateral sclerosis (ALS) is the presence of intracellular inclusions in degenerating neurons and their axons. The appearance and localization of these pathological structures depend on an aggregated protein that forms their scaffold. We investigated if γ-synuclein, an aggregation-prone protein highly expressed in healthy motor neurons, and predominantly localized in their axons and synaptic terminals is involved in ALS pathology.

Methods

Immunostaining of histological sections and sequential protein extraction from postmortem neural samples followed by immunoblotting.

Results

Immunohistochemical screening revealed a subset of sporadic (9 of 31) and familial (8 of 23) ALS cases with a novel type of pat

SUBMITTER: Peters OM 

PROVIDER: S-EPMC4301672 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature