title: Is involvement of inflammation underestimated in Pelizaeus-Merzbacher disease?
abstract: 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.
authors.affiliation: INSERM, U1127, F-75013, Paris, France.|CNRS, UMR 7225, F-75013, Paris, France.|Université Pierre et Marie Curie-Paris 6, UMR_S 1127, F-75013, Paris, France.|Institut du Cerveau et de la Moelle épinière, F-75013, Paris, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Marteyn
authors.affiliation: INSERM, U1127, F-75013, Paris, France. anne.baron@upmc.fr.|CNRS, UMR 7225, F-75013, Paris, France. anne.baron@upmc.fr.|Université Pierre et Marie Curie-Paris 6, UMR_S 1127, F-75013, Paris, France. anne.baron@upmc.fr.|Institut du Cerveau et de la Moelle épinière, F-75013, Paris, France. anne.baron@upmc.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: Baron-Van Evercooren
mesh_terms: D006801:Humans; D007249:Inflammation / Q000473:pathology* / Q000628:therapy; D018991:Myelin Proteolipid Protein / Q000235:genetics; D009186:Myelin Sheath / Q000473:pathology; D020371:Pelizaeus-Merzbacher Disease / Q000473:pathology* / Q000628:therapy
chemical_list: D018991:Myelin Proteolipid Protein
keywords: Pelizaeus-Merzbacher disease (PMD); cell-based therapy; modulation of inflammation; neuroinflammation; remyelination