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Is involvement of inflammation underestimated in Pelizaeus–Merzbacher disease?
Author(s) -
Marteyn Antoine,
BaronVan Evercooren Anne
Publication year - 2016
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.23931
Subject(s) - neuroinflammation , microglia , astrogliosis , leukodystrophy , multiple sclerosis , inflammation , neuroscience , myelin , oligodendrocyte , biology , progenitor cell , immunology , medicine , disease , stem cell , central nervous system , pathology , genetics
Pelizaeus–Merzbacher disease (PMD) is a severe hypomyelinating leukodystrophy resulting from proteolipid protein 1 gene ( PLP1 ) mutations leading to oligodendrocyte loss. While neuroinflammation has recently become a common feature and actor in neurodegenerative diseases, the involvement of inflammation in PMD physiopathology is still highly debated despite evidence for strong astrogliosis and microglial cell activation. Activation of the innate immune system, and more particularly, of microglia and astrocytes, is mostly associated with the deleterious role of neuroinflammation. However, in diseases such as multiple sclerosis, microglia appear beneficial for repair based on their role in myelin debris removal or recruitment and differentiation of oligodendrocyte progenitor cells. In this review, we will discuss recent published data in terms of their relevance to the role of microglia in PMD evolution, and of their impact on the improvement of therapeutic approaches combining immunomodulation and cell therapy to promote optimal recovery. © 2016 Wiley Periodicals, Inc.