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), 159-166. (doi:10.1016/j.molmed.2006.02.006).


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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)
Related URLs:
Subjects: R Medicine > RB Pathology
R Medicine > RC Internal medicine > RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry
Q Science > QH Natural history > QH301 Biology
Divisions : University Structure - Pre August 2011 > School of Biological Sciences
ePrint ID: 56788
Accepted Date and Publication Date:
April 2006Published
Date Deposited: 08 Aug 2008
Last Modified: 31 Mar 2016 12:37

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