Defective translation initiation causes vanishing of cerebral white matter


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).

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Description/Abstract

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.

Item Type: Article
Digital Object Identifier (DOI): doi:10.1016/j.molmed.2006.02.006
ISSNs: 1471-4914 (print)
Subjects:

ePrint ID: 56788
Date :
Date Event
April 2006Published
Date Deposited: 08 Aug 2008
Last Modified: 16 Apr 2017 17:40
Further Information:Google Scholar
URI: http://eprints.soton.ac.uk/id/eprint/56788

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