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Molecular mechanisms of inherited demyelinating neuropathies
Author(s) -
Scherer Steven S.,
Wrabetz Lawrence
Publication year - 2008
Publication title -
glia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.954
H-Index - 164
eISSN - 1098-1136
pISSN - 0894-1491
DOI - 10.1002/glia.20751
Subject(s) - biology , myelin , phenotype , demyelinating disorder , neuroscience , molecular genetics , gene , identification (biology) , genetics , central nervous system , botany
The past 15 years have witnessed the identification of more than 25 genes responsible for inherited neuropathies in humans, many associated with primary alterations of the myelin sheath. A remarkable body of work in patients, as well as animal and cellular models, has defined the clinical and molecular genetics of these illnesses and shed light on how mutations in associated genes produce the heterogeneity of dysmyelinating and demyelinating phenotypes. Here, we review selected recent developments from work on the molecular mechanisms of these disorders and their implications for treatment strategies. © 2008 Wiley‐Liss, Inc.