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[Neuronal intermediate filament proteins]

O Bousquet1, M M Portier

  • 1Laboratoire de Biochimie Cellulaire, Collège de France, Paris, France.

Comptes Rendus Des Seances De La Societe De Biologie Et De Ses Filiales
|January 1, 1996
PubMed
Summary
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This review covers intermediate filament proteins in neuron development, focusing on peripherin for axonal guidance and neurofilament proteins (NFP) for axonal transport. NFP disruptions are linked to motor neuron diseases, with transgenic models aiding research.

Area of Science:

  • Neuroscience
  • Cell Biology
  • Developmental Biology

Context:

  • Intermediate filament proteins are crucial for neuronal structure and function.
  • Seven such proteins are expressed during neuronal development.
  • Neurofilament proteins (NFP) and peripherin are the most studied.

Purpose:

  • To review the characteristics and roles of neuronal intermediate filament proteins.
  • To highlight the distinct functions of peripherin and NFP in neuronal development and disease.

Summary:

  • Peripherin's plasticity is key for axonal navigation during development and regeneration.
  • NFP maintain axonal caliber and transport, with disruptions linked to motor neuron diseases.
  • Transgenic and overexpressed NFP mouse models are utilized for studying these diseases.

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Impact:

  • Understanding these proteins aids in comprehending neuronal development and regeneration processes.
  • Insights into NFP's role in motor neuron diseases can inform therapeutic strategies.
  • The reviewed models offer valuable tools for preclinical research in neurodegenerative disorders.