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Intra-axonal protein aggregation in the peripheral nervous system.

Anne Vital1, Wassillios G Meissner, Marie-Hélène Canron

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Journal of the Peripheral Nervous System : JPNS
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PubMed
Summary

Intracellular protein aggregates, like ubiquitin and tau, are found in chronic axonal peripheral neuropathy (PN). Ubiquitin aggregates correlate with reduced axonal transport, suggesting a role in neurodegenerative disorders.

Keywords:
axonopathyperipheral nerve biopsyprotein aggregationtauubiquitin

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Cell Biology

Background:

  • Intracellular protein aggregates are key features of neurodegenerative diseases.
  • Defects in axonal transport are implicated in neurodegeneration.
  • Peripheral neuropathy (PN) can involve axonal damage.

Purpose of the Study:

  • To investigate intra-axonal protein aggregation and axonopathy in patients with chronic axonal PN of unknown etiology.
  • To examine the relationship between protein aggregates and axonal transport defects.

Main Methods:

  • Immunohistochemistry and electron microscopy were performed on peripheral nerve biopsies from 12 patients.
  • Patients included three with idiopathic Parkinson's disease (PD) and nine without PD.
  • Analysis focused on ubiquitin, tau (AT8), and phosphorylated α-synuclein aggregates.

Main Results:

  • Ubiquitin aggregates were more prevalent in PD patients.
  • Tau AT8 aggregates were found in five non-PD patients.
  • Enlarged axons with organelle crowding, indicating slowed axonal transport, were observed in six patients, including all PD cases.
  • Ubiquitin aggregate load correlated with reduced axonal flux, unlike tau or 14-3-3 β.

Conclusions:

  • Both ubiquitin and abnormal tau intra-axonal aggregates can be present in chronic axonal PN.
  • Ubiquitin aggregates may contribute to reduced axonal flux or be a consequence of impaired axonal transport.
  • The findings highlight the role of protein aggregation in axonal dysfunction in PN.