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A ffected astrocytes in the spinal cord of the leukodystrophy vanishing white matter
Author(s) -
Leferink Prisca S.,
Breeuwsma Nicole,
Bugiani Marianna,
van der Knaap Marjo S.,
Heine Vivi M.
Publication year - 2018
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.23289
Subject(s) - spinal cord , white matter , leukodystrophy , astrocyte , spasticity , neuroscience , myelin , oligodendrocyte , pathology , ataxia , biology , multiple sclerosis , leukoencephalopathy , spinal cord injury , central nervous system , disease , medicine , magnetic resonance imaging , immunology , physical medicine and rehabilitation , radiology
Abstract Leukodystrophies are often devastating diseases, presented with progressive clinical signs as spasticity, ataxia and cognitive decline, and lack proper treatment options. New therapy strategies for leukodystrophies mostly focus on oligodendrocyte replacement to rescue lack of myelin in the brain, even though disease pathology also often involves other glial cells and the spinal cord. In this study we investigated spinal cord pathology in a mouse model for Vanishing White Matter disease (VWM) and show that astrocytes in the white matter are severely affected. Astrocyte pathology starts postnatally in the sensory tracts, followed by changes in the astrocytic populations in the motor tracts. Studies in post‐mortem tissue of two VWM patients, a 13‐year‐old boy and a 6‐year‐old girl, confirmed astrocyte abnormalities in the spinal cord. For proper development of new treatment options for VWM and, possibly, other leukodystrophies, future studies should investigate spinal cord involvement.