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Regulation of protein synthesis in lymphoblasts from vanishing white matter patients

Kollenburg, Barbara, Thomas, Adri A.M., Vermeulen, Gerre, Bertrand, Gesina A.M., Berkel, Carola G.M., Pronk, Jan C., Proud, Christopher G., Knaap, Marjo S. and Scheper, Gert C. (2006) Regulation of protein synthesis in lymphoblasts from vanishing white matter patients Neurobiology of Disease, 21, (3), pp. 496-504. (doi:10.1016/j.nbd.2005.08.009).

Record type: Article


Leukoencephalopathy with vanishing white matter (VWM) is an inherited childhood white matter disorder, caused by mutations in the genes encoding eukaryotic initiation factor 2B (eIF2B). The present study showed that, while the eIF2B activity was reduced in VWM lymphoblasts, the expression levels of the eIF2B subunits were similar to control lymphoblast lines. The mutations in eIF2B did not affect the interaction with eIF2. Strikingly, no apparent differences for the regulation of protein synthesis, measured by [35S]-methionine incorporation, were found between control and VWM lymphoblasts. Western blotting showed that, in some VWM cells, exposure to heat shock caused a decrease in the expression of specific eIF2B subunits. Most importantly, the increase in phosphorylation of eIF2? in response to heat shock was lower in VWM lymphoblasts than in control cells. These findings could form part of the explanation for the episodes of rapid and severe deterioration in VWM patients that are precipitated by febrile infections.

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Published date: March 2006
Keywords: translation, white matter, eIF2B, heat shock, leukoencephalopathy, childhood


Local EPrints ID: 56396
ISSN: 0969-9961
PURE UUID: 09b56d64-a0a1-46ed-a36d-8f9a8e9423ca

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Date deposited: 07 Aug 2008
Last modified: 17 Jul 2017 14:31

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Author: Barbara Kollenburg
Author: Adri A.M. Thomas
Author: Gerre Vermeulen
Author: Gesina A.M. Bertrand
Author: Carola G.M. Berkel
Author: Jan C. Pronk
Author: Christopher G. Proud
Author: Marjo S. Knaap
Author: Gert C. Scheper

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