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Cytoskeleton of the Nervous System pp 261–277Cite as

Knockout Models of Neurofilament Proteins

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Part of the book series: Advances in Neurobiology ((NEUROBIOL,volume 3))

Abstract

Neurofilaments (NFs) are the most prominent cytoskeleton components of large myelinated axons from adult central and peripheral nervous systems. In the last 15 years, the gene targeting technique has been widely used to investigate the role of NF proteins in neuronal function. Gene knockout studies have demonstrated that NFs are crucial to expand the caliber of myelinated axons and consequently to increase their conduction velocity. However, the mechanism by which NFs determine the axonal diameter is not yet fully elucidated. NFs also contribute to the dynamic properties of the axonal cytoskeleton during neuronal differentiation, axon outgrowth and regeneration. Perturbations of their metabolism and organization are frequently associated with neurodegenerative disorders, including amyotrophic lateral sclerosis, Charcot-Marie-Tooth disease, Alzheimer’s disease and giant axonal neuropathy. Here, we describe how mouse knockout models of NF proteins have been used to study the multiple aspects of NF biology.

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Acknowledgments

J.-P.J. holds a Canada Research Chair in neurodegeneration and R.P. is the recipient of the Tim E. Noël Fellowship (ALS Society of Canada).

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Authors and Affiliations

  1. Department of Psychiatry and Neuroscience, Laval University, Research Centre of CHUQ, 2705, boulevard Laurier, T-2-50, Québec (Québec), Canada

    Rodolphe Perrot

  2. Department of Anatomy and Physiology, Centre de Recherche du Centre Hospitalier, Universitaire de Québec, Laval University, Sainte-Foy, QC, Canada, G1V 4G2

    Jean-Pierre Julien

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  1. Rodolphe Perrot
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  2. Jean-Pierre Julien
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Correspondence to Jean-Pierre Julien .

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Editors and Affiliations

  1. Psychiatric Research, Nathan S. Kline Institute for, 140 Old Orangeburg Road, Orangeburg, 10962, New York, USA

    Ralph A. Nixon

  2. Psychiatric Research, Nathan S. Kline Institute for, 140 Old Orangeburg Road, Orangeburg, 10962, New York, USA

    Aidong Yuan

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Perrot, R., Julien, JP. (2011). Knockout Models of Neurofilament Proteins. In: Nixon, R., Yuan, A. (eds) Cytoskeleton of the Nervous System. Advances in Neurobiology, vol 3. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6787-9_12

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