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Myelin Pathology: Involvement of Molecular Chaperones and the Promise of Chaperonotherapy

1
Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University of Palermo, 90127 Palermo, Italy
2
Euro-Mediterranean Institute of Science and Technology (IEMEST), 90139 Palermo, Italy
3
Department of Microbiology and Immunology, School of Medicine, University of Maryland at Baltimore-Institute of Marine and Environmental Technology (IMET), Baltimore, MD 21202, USA
*
Author to whom correspondence should be addressed.
Brain Sci. 2019, 9(11), 297; https://doi.org/10.3390/brainsci9110297
Received: 26 September 2019 / Revised: 23 October 2019 / Accepted: 27 October 2019 / Published: 30 October 2019
(This article belongs to the Collection Collection on Clinical Neuroscience)
The process of axon myelination involves various proteins including molecular chaperones. Myelin alteration is a common feature in neurological diseases due to structural and functional abnormalities of one or more myelin proteins. Genetic proteinopathies may occur either in the presence of a normal chaperoning system, which is unable to assist the defective myelin protein in its folding and migration, or due to mutations in chaperone genes, leading to functional defects in assisting myelin maturation/migration. The latter are a subgroup of genetic chaperonopathies causing demyelination. In this brief review, we describe some paradigmatic examples pertaining to the chaperonins Hsp60 (HSPD1, or HSP60, or Cpn60) and CCT (chaperonin-containing TCP-1). Our aim is to make scientists and physicians aware of the possibility and advantages of classifying patients depending on the presence or absence of a chaperonopathy. In turn, this subclassification will allow the development of novel therapeutic strategies (chaperonotherapy) by using molecular chaperones as agents or targets for treatment. View Full-Text
Keywords: myelin; myelin pathology; myelinopathies; proteinopathies; chaperonopathies; Hsp60; CCT; chaperonotherapy myelin; myelin pathology; myelinopathies; proteinopathies; chaperonopathies; Hsp60; CCT; chaperonotherapy
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Scalia, F.; Marino Gammazza, A.; Conway de Macario, E.; Macario, A.J.L.; Cappello, F. Myelin Pathology: Involvement of Molecular Chaperones and the Promise of Chaperonotherapy. Brain Sci. 2019, 9, 297.

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