Similarities between inherited demyelinating neuropathies and Wallerian degeneration: an old repair program may cause myelin and axon perturbation under nonlesion conditions.
Am J Pathol
; 183(3): 655-60, 2013 Sep.
Article
em En
| MEDLINE
| ID: mdl-23831295
ABSTRACT
Wallerian degeneration (WD) and inherited demyelinating neuropathies of the Charcot-Marie-Tooth type 1 (CMT1) appear to represent completely distinct events. CMT1-like diseases are chronic disorders of peripheral nerves that are genetically caused and lead to secondary neurodegenerative events, resulting in usually non-treatable disabilities, whereas WD is an acute, usually transient, reaction on injuries, aiming to allow peripheral nerve regeneration. Despite these differences, there are some striking similarities regarding molecular characteristics of neural cells in the affected peripheral nerves. The most conspicuous similarities might comprise the inflammatory component in both situations, as identified in appropriate mouse models. However, although inflammation is a beneficial component in WD, leading to removal of regrowth-repellent myelin debris, inflammation in CMT1 mouse models causes damage of initially intact nerve fibers. We hypothesize that, in CMT1 models, molecular pathways are activated that are shared with an important repair program after peripheral nerve injury, but lead to neural perturbation when activated under nonlesion conditions, as is the case in CMT1. These novel insights into the pathogenesis of CMT1 might be instrumental for the development of new therapeutic options in humans.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Axônios
/
Degeneração Walleriana
/
Doenças Desmielinizantes
/
Doenças Genéticas Inatas
/
Bainha de Mielina
/
Regeneração Nervosa
Tipo de estudo:
Prognostic_studies
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Am J Pathol
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Alemanha