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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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 |
|---|---|
| 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 |
| Item ID: | 56788 |
| Date Deposited: | 08 Aug 2008 |
| Last Modified: | 12 May 2013 01:18 |
| Contributors: | Scheper, Gert C. (Author) Proud, Christopher G. (Author) van der Knaap, Marjo S. (Author) |
| Date: | April 2006 |
| Status: | Published |
| URI: | http://eprints.soton.ac.uk/id/eprint/56788 |
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