Molecular Neurobiology

, Volume 38, Issue 1, pp 27-65

First online:

Review of the Multiple Aspects of Neurofilament Functions, and their Possible Contribution to Neurodegeneration

  • Rodolphe PerrotAffiliated withCentre de Recherche du Centre Hospitalier Universitaire de Québec, Department of Anatomy and Physiology of Laval University
  • , Raphael BergesAffiliated withLaboratoire de Neurobiologie and Transgenese, UPRES-EA3143, INSERM IFR132
  • , Arnaud BocquetAffiliated withDivision des Maladies Cardiovasculaires II, Centre de Recherche Pierre Fabre
  • , Joel EyerAffiliated withLaboratoire de Neurobiologie and Transgenese, UPRES-EA3143, INSERM IFR132 Email author 

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Neurofilaments (NF) are the most abundant cytoskeletal component of large myelinated axons from adult central and peripheral nervous system. Here, we provide an overview of the complementary approaches, including biochemistry, cell biology and transgenic technology that were used to investigate the assembly, axonal transport and functions of NF in normal and pathological situations. Following their synthesis and assembly in the cell body, NFs are transported along the axon. This process is finely regulated via phosphorylation of the carboxy-terminal part of the two high-molecular-weight subunits of NF. The correct formation of an axonal network of NF is crucial for the establishment and maintenance of axonal calibre and consequently for the optimisation of conduction velocity. The frequent disorganisation of NF network observed in several neuropathologies support their contribution. However, despite the presence of NF mutations found in some patients, the exact relations between these mutations, the abnormal NF organisation and the pathological process remain a challenging field of investigation.


Neurofilament Intermediate filaments Cytoskeleton Axonal transport Neurodegenerative diseases