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Gamma-synuclein pathology in amyotrophic lateral sclerosis
Owen M Peters1, Tatyana Shelkovnikova1, John Robin Highley2
1School of Biosciences, Cardiff University Museum Avenue, Cardiff, CF10 3AX, United Kingdom.
Annals of Clinical and Translational Neurology
|February 3, 2015
Summary
Pathological aggregation of gamma-synuclein (γ-synuclein) was observed in a subset of amyotrophic lateral sclerosis (ALS) cases. This finding suggests γ-synuclein aggregation may contribute to ALS pathogenesis.
Area of Science:
- Neuroscience
- Neuropathology
- Molecular Biology
Background:
- Amyotrophic lateral sclerosis (ALS) is characterized by motor neuron degeneration.
- Intracellular inclusions are a hallmark of ALS pathology.
- The protein scaffold forming these inclusions is critical for their structure.
Purpose of the Study:
- To investigate the potential role of γ-synuclein in ALS pathology.
- γ-synuclein is an aggregation-prone protein highly expressed in motor neurons.
Main Methods:
- Immunohistochemistry on postmortem neural sections.
- Sequential protein extraction and immunoblotting from spinal cord tissues.
Main Results:
- γ-synuclein accumulation found in distinct profiles in the dorsolateral column of ALS cases.
- Detergent-insoluble γ-synuclein species detected in corticospinal tracts of an ALS patient.
- These profiles differ from known ALS inclusion markers and may represent degenerated axons.
Conclusions:
- Pathological aggregation of γ-synuclein is implicated in ALS pathogenesis.
- γ-synuclein may represent a novel target for ALS research.
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