論文

査読有り
2017年7月

A longitudinal DTI and histological study of the spinal cord reveals early pathological alterations in G93A-SOD1 mouse model of amyotrophic lateral sclerosis

EXPERIMENTAL NEUROLOGY
  • Stefania Marcuzzo
  • ,
  • Silvia Bonanno
  • ,
  • Matteo Figini
  • ,
  • Alessandro Scotti
  • ,
  • Ileana Zucca
  • ,
  • Ludovico Minati
  • ,
  • Nilo Riva
  • ,
  • Teuta Domi
  • ,
  • Andrea Fossaghi
  • ,
  • Angelo Quattrini
  • ,
  • Barbara Galbardi
  • ,
  • Sara D'Alessandro
  • ,
  • Maria Grazia Bruzzone
  • ,
  • Jose Manuel Garcia-Verdugo
  • ,
  • Victoria Moreno-Manzano
  • ,
  • Renato Mantegazza
  • ,
  • Pia Bernasconi

293
開始ページ
43
終了ページ
52
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1016/j.expneurol.2017.03.018
出版者・発行元
ACADEMIC PRESS INC ELSEVIER SCIENCE

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective motor neuron degeneration in the motor cortex, brainstem and spinal cord. It is generally accepted that ALS is caused by death of motor neurons, however the exact temporal cascade of degenerative processes is not yet completely known. To identify the early pathological changes in spinal cord of G93A-SOD1 AIS mice we performed a comprehensive longitudinal analysis employing diffusion-tensor magnetic resonance imaging alongside histology and electron microscopy, in parallel with peripheral nerve histology. We showed the gradient of degeneration appearance in spinal cord white and gray matter, starting earliest in the ventral white matter, due to a cascade of pathological events including axon dysfunction and mitochondrial changes. Notably, we found that even the main sensory regions are affected by the neurodegenerative process at symptomatic disease phase. Overall our results attest the applicability of DTI in determining disease progression in ALS mice. These findings suggest that DTI could be potentially adapted in humans to aid the assessment of ALS progression and eventually the evaluation of treatment efficacy. (C) 2017 Elsevier Inc. All rights reserved.

Web of Science ® 被引用回数 : 13

リンク情報
DOI
https://doi.org/10.1016/j.expneurol.2017.03.018
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000401784300004&DestApp=WOS_CPL
URL
http://www.scopus.com/inward/record.url?eid=2-s2.0-85016473519&partnerID=MN8TOARS
URL
http://orcid.org/0000-0002-2532-1674

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