Activation of the heat shock response in familial amyloidotic polyneuropathy

Journal of Neuropathology and Experimental Neurology
Sofia D SantosMaria João Saraiva

Abstract

The heat shock proteins (Hsps) have been implicated in a variety of neurodegenerative diseases in which the underlying pathology is protein aggregation. Here, we studied the heat shock response in familial amyloidotic polyneuropathy (FAP), a neurodegenerative disease caused by aggregation and extracellular tissue deposition of mutated transthyretin (TTR). We observed greater expression of Hsp27 and Hsp70 related to the presence of extracellular TTR aggregates in human FAP nerve, skin, and salivary gland biopsies than in normal controls. Transthyretin aggregates did not colocalize with Hsp, suggesting that extracellular TTR tissue deposits induce an intracellular stress response. Moreover, the heat shock transcription factor 1 was upregulated and localized to nuclei in affected tissues. Transgenic mice expressing the V30M mutant form of TTR similarly showed the presence of TTR deposits, induced activation of heat shock transcription factor 1, and increased synthesis of Hsp. Furthermore, the addition of toxic TTR aggregates to cultures of human and rodent neuroblastoma cell lines induced upregulation of Hsp70 and Hsp27. Taken together, these novel findings suggest new avenues for research on pathogenic mechanisms in FAP and ident...Continue Reading

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Citations

Mar 1, 2012·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Joel N BuxbaumDaniel R Salomon
Mar 5, 2013·Annual Review of Biochemistry·Luis M Blancas-Mejía, Marina Ramirez-Alvarado
Jul 24, 2012·FEBS Letters·Maria Rosário Almeida, Maria João Saraiva
Nov 16, 2011·Journal of Neuropathology and Experimental Neurology·Joana Magalhães, Maria João Saraiva
Sep 2, 2010·International Journal of Experimental Pathology·Joana MagalhãesMaria João Saraiva
Oct 20, 2018·Journal of Cell Science·Christoph NiemietzHartmut H-J Schmidt
Feb 9, 2017·Annals of Clinical and Translational Neurology·Diana SantosAlda Sousa

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