AUTHOR=Barton Samantha K. , Gregory Jenna M. , Selvaraj Bhuvaneish T. , McDade Karina , Henstridge Christopher M. , Spires-Jones Tara L. , James Owen G. , Mehta Arpan R. , Story David , Burr Karen , Magnani Dario , Isaacs Adrian M. , Smith Colin , Chandran Siddharthan TITLE=Dysregulation in Subcellular Localization of Myelin Basic Protein mRNA Does Not Result in Altered Myelination in Amyotrophic Lateral Sclerosis JOURNAL=Frontiers in Neuroscience VOLUME=15 YEAR=2021 URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2021.705306 DOI=10.3389/fnins.2021.705306 ISSN=1662-453X ABSTRACT=
Pathological hallmarks of amyotrophic lateral sclerosis (ALS), including protein misfolding, are well established in oligodendrocytes. More recently, an RNA trafficking deficit of key myelin proteins has been suggested in oligodendrocytes in ALS but the extent to which this affects myelination and the relative contribution of this to disease pathogenesis is unclear. ALS autopsy research findings showing demyelination contrasts with the routine clinical-pathological workup of ALS cases where it is rare to see white matter abnormalities other than simple Wallerian degeneration secondary to widespread neuronal loss. To begin to address this apparent variance, we undertook a comprehensive evaluation of myelination at an RNA, protein and structural level using human pathological material from sporadic ALS patients, genetic ALS patients (harboring