Defective translation initiation causes vanishing of cerebral white matter
Defective translation initiation causes vanishing of cerebral white matter
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.
159-166
Scheper, Gert C.
1ed78a31-c592-4ea4-8256-a75ad241d802
Proud, Christopher G.
59dabfc8-4b44-4be8-a17f-578a58550cb3
van der Knaap, Marjo S.
b46a8973-a464-464c-a35d-5e0c600749c3
April 2006
Scheper, Gert C.
1ed78a31-c592-4ea4-8256-a75ad241d802
Proud, Christopher G.
59dabfc8-4b44-4be8-a17f-578a58550cb3
van der Knaap, Marjo S.
b46a8973-a464-464c-a35d-5e0c600749c3
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), .
(doi:10.1016/j.molmed.2006.02.006).
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.
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Published date: April 2006
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Local EPrints ID: 56788
URI: http://eprints.soton.ac.uk/id/eprint/56788
ISSN: 1471-4914
PURE UUID: 7be9302f-680c-43f2-bc8f-5ddceda8c5d2
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Date deposited: 08 Aug 2008
Last modified: 15 Mar 2024 11:03
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Author:
Gert C. Scheper
Author:
Christopher G. Proud
Author:
Marjo S. van der Knaap
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