Oxidative stress and mitochondrial dynamics malfunction are linked in P elizaeus‐ M erzbacher disease

Volume: 28, Issue: 5, Pages: 611 - 630
Published: Dec 26, 2017
Abstract
Pelizaeus-Merzbacher disease (PMD) is a fatal hypomyelinating disorder characterized by early impairment of motor development, nystagmus, choreoathetotic movements, ataxia and progressive spasticity. PMD is caused by variations in the proteolipid protein gene PLP1, which encodes the two major myelin proteins of the central nervous system, PLP and its spliced isoform DM20, in oligodendrocytes. Large duplications including the entire PLP1 gene are...
Paper Details
Title
Oxidative stress and mitochondrial dynamics malfunction are linked in P elizaeus‐ M erzbacher disease
Published Date
Dec 26, 2017
Volume
28
Issue
5
Pages
611 - 630
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