Mutation of the extracellular domain of tumour necrosis factor receptor 1 causes reduced NF-κB activation due to decreased surface expression

Siebert, S. , Fielding, C. A., Williams, B. D. and Brennan, P. (2005) Mutation of the extracellular domain of tumour necrosis factor receptor 1 causes reduced NF-κB activation due to decreased surface expression. FEBS Letters, 579(23), pp. 5193-5198. (doi:10.1016/j.febslet.2005.08.037) (PMID:16162344)

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Abstract

Tumour necrosis factor receptor-associated periodic syndrome (TRAPS) results from point mutations in the extracellular domain of TNF receptor 1 (TNFRSF1A), but the effects of the mutations are controversial. This study shows that reduced NF-κB signalling is a feature of four TRAPS mutations. Reduced signalling correlates with reduced surface expression, measured by flow cytometry and microscopy. This suggests that correct formation of the extracellular domain of TNFRSF1A is important for localisation and receptor function. Importantly, our data provides a mechanism for the reduced TNFRSF1 signalling observed in a patient cell line.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Siebert, Dr Stefan
Authors: Siebert, S., Fielding, C. A., Williams, B. D., and Brennan, P.
College/School:College of Medical Veterinary and Life Sciences > Institute of Infection Immunity and Inflammation
Journal Name:FEBS Letters
Publisher:Elsevier BV
ISSN:0014-5793
ISSN (Online):1873-3468

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