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Defective translation initiation causes vanishing of cerebral white matter

Record type: Article

Leukoencephalopathy with vanishing white matter (VWM) is one of the most prevalent inherited white-matter disorders, especially in Caucasian populations. VWM is unusual because of its sensitivity to febrile infections and minor head trauma. The basic defect of this enigmatic brain disease resides in the regulation of initiation of protein synthesis. Recently, undue activation of the unfolded-protein response has emerged as an important factor in the pathophysiology of VWM. Here, we discuss the mechanisms that might be responsible for the selective involvement of the brain white matter in VWM. At present, VWM research is in need of an animal model to study disease mechanisms and therapeutic interventions.

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Citation

Scheper, Gert C., Proud, Christopher G. and van der Knaap, Marjo S. (2006) Defective translation initiation causes vanishing of cerebral white matter Trends in Molecular Medicine, 12, (4), pp. 159-166. (doi:10.1016/j.molmed.2006.02.006).

More information

Published date: April 2006

Identifiers

Local EPrints ID: 56788
URI: http://eprints.soton.ac.uk/id/eprint/56788
ISSN: 1471-4914
PURE UUID: 7be9302f-680c-43f2-bc8f-5ddceda8c5d2

Catalogue record

Date deposited: 08 Aug 2008
Last modified: 17 Jul 2017 14:30

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Contributors

Author: Gert C. Scheper
Author: Christopher G. Proud
Author: Marjo S. van der Knaap

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