TY - JOUR
T1 - Myelin pathology: Involvement of molecular chaperones and the promise of chaperonotherapy
AU - Scalia, Federica
AU - Marino Gammazza, Antonella
AU - Cappello, Francesco
AU - Gammazza, Antonella Marino
AU - Scalia, Federica
AU - De Macario, Everly Conway
AU - Cappello, Francesco
AU - De Macario, Everly Conway
AU - Macario, Alberto J. L.
AU - Macario, Alberto J.L.
PY - 2019
Y1 - 2019
N2 - 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.
AB - 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.
UR - http://hdl.handle.net/10447/397365
UR - https://www.mdpi.com/2076-3425/9/11/297/pdf
M3 - Article
VL - 9
SP - 297-
JO - Brain Sciences
JF - Brain Sciences
SN - 2076-3425
ER -