Affected astrocytes in the spinal cord of the leukodystrophy vanishing white matter

Glia
Prisca S LeferinkVivi M Heine

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.

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Citations

May 28, 2019·Neuromolecular Medicine·Melisa HerreroOrna Elroy-Stein
Sep 15, 2019·The FEBS Journal·Holly A Rutherford, Noémie Hamilton
Oct 7, 2019·Journal of Neurochemistry·Mika Terumitsu-TsujitaHironaka Igarashi
Aug 4, 2018·Case Reports in Pediatrics·D HettiaracchchiV H W Dissanayake
Aug 23, 2019·Annals of Neurology·Prisca S LeferinkVivi M Heine
Dec 9, 2021·Acta Neuropathologica·Marianna BugianiMarjo S van der Knaap

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Datasets Mentioned

BETA
AB5603

Methods Mentioned

BETA
genotyping
single cell sequencing

Software Mentioned

SPSS Statistics
Adobe Photoshop
Image J

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