Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy - Soler-Martín - 2012 - Neuropathology and Applied Neurobiology - Wiley Online Library

Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy - Soler-Martín - 2012 - Neuropathology and Applied Neurobiology - Wiley Online Library

Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy

1. C. Soler-Martín¹,
2. Ú. Vilardosa¹,
3. S. Saldaña-Ruíz¹,
4. N. Garcia²,
5. J. Llorens^1,*

Article first published online: 6 JAN 2012

DOI: 10.1111/j.1365-2990.2011.01178.x

© 2011 The Authors. Neuropathology and Applied Neurobiology © 2011 British Neuropathological Society

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Neuropathology and Applied Neurobiology

Volume 38, Issue 1, pages 61–71, February 2012

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How to Cite

Soler-Martín, C., Vilardosa, Ú., Saldaña-Ruíz, S., Garcia, N. and Llorens, J. (2012), Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy. Neuropathology and Applied Neurobiology, 38: 61–71. doi: 10.1111/j.1365-2990.2011.01178.x

Author Information

1. ¹ Departament de Ciències Fisiològiques II, Universitat de Barcelona, and Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), Hospitalet de Llobregat
2. ² Unitat d'Histologia i Neurobiologia (UHN), Facultat de Medicina i Ciències de la Salut, Universitat Rovira i Virgili, Reus, Spain

^*Jordi Llorens, Departament de Ciències Fisiològiques II, Universitat de Barcelona, and Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), 08907 Hospitalet de Llobregat, Spain. Tel: +34 93 402 4277; Fax: +34 93 402 4268; E-mail: jllorens@ub.edu

Publication History

1. Issue published online: 6 JAN 2012
2. Article first published online: 6 JAN 2012
3. Accepted manuscript online: 31 MAR 2011 03:42AM EST
4. Received 13 January 2011; Accepted after revision 25 March 2011; Published online Article Accepted on 31 March 2011

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Keywords:

• 3,3′-iminodipropionitrile;
• amyotrophic lateral sclerosis;
• axonal transport;
• neurofilament proteins;
• motor terminals;
• rat

C. Soler-Martín, Ú. Vilardosa, S. Saldaña-Ruíz, N. Garcia and J. Llorens (2012) Neuropathology and Applied Neurobiology38, 61–71

Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy

Aims: Rodents exposed to 3,3′-iminodipropionitrile (IDPN) develop an axonopathy similar to that observed in amyotrophic lateral sclerosis motor neurones, in which neurofilaments accumulate in swollen proximal axon segments. This study addressed the hypotheses that this proximal axonopathy is associated with loss of neurofilament proteins in the neuromuscular junctions and a progressive loss of neurofilaments advancing in a distal-proximal direction from the distal motor nerve. Methods: Adult male Long-Evans rats were exposed to 0 or 15 mM of IDPN in drinking water for 1, 3 or 5 weeks, and their distal axons and neuromuscular junction organization studied by immunohistochemistry. Quantitative data were obtained by confocal microscopy on whole mounts of the Levator auris longus. Results: Muscles showed no change in the distribution of acetylcholine receptor labelling in the neuromuscular junctions after IDPN. In contrast, the amount of neurofilament labelling in the junctions was significantly reduced by IDPN, assessed with two different anti-neurofilament antibodies. In preterminal axons and in more proximal axon levels, no statistically significant reductions in neurofilament content were observed. Conclusions: The proximal neurofilamentous axonopathy induced by IDPN is associated with an abnormally low content of neurofilaments in the motor terminals, with a potential impact in the function or stability of the neuromuscular junction. In contrast, neurofilaments are significantly maintained in the distal axon.

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