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Hsp90 inhibition protects against inherited retinal degeneration

Autor
Aguilà, M.; Bevilacqua, D.; McCulley, C.; Schwarz, N.; Athanasiou, D.; Kanuga, N.; Novoselov, S.; Lange, C.; Ali, R.; Bainbridge, J.; Gias, C.; Coffey, P.; Garriga, P.; Cheetham, M.
Tipus d'activitat
Article en revista
Revista
Human molecular genetics
Data de publicació
2014-04-15
Volum
23
Número
8
Pàgina inicial
2164
Pàgina final
2175
DOI
https://doi.org/10.1093/hmg/ddt613 Obrir en finestra nova
Repositori
http://hdl.handle.net/2117/22886 Obrir en finestra nova
URL
http://www.ncbi.nlm.nih.gov/pubmed/24301679 Obrir en finestra nova
Resum
The molecular chaperone Hsp90 is important for the functional maturation of many client proteins, and inhibitors are in clinical trials for multiple indications in cancer. Hsp90 inhibition activates the heat shock response and can improve viability in a cell model of the P23H misfolding mutation in rhodopsin that causes autosomal dominant retinitis pigmentosa (adRP). Here, we show that a single low dose of the Hsp90 inhibitor HSP990 enhanced visual function and delayed photoreceptor degeneration...
Citació
Aguilà, M. [et al.]. Hsp90 inhibition protects against inherited retinal degeneration. "Human molecular genetics", 15 Abril 2014, vol. 23, núm. 8, p. 2164-2175.
Paraules clau
Dominant retinitis-pigmentosa, Gene, Heat-shock response, Kinase, Leber congenital amaurosis, Mouse model, Mutant rhodopsin CGMP Phosphediesterase, Quality-control, Rhodopsin-arrestin complexes
Grup de recerca
CEBIM - Centre de Biotecnologia Molecular
GBMI - Grup de Biotecnologia Molecular i Industrial

Participants

  • Aguilà Cerdà, Mònica  (autor)
  • Bevilacqua, Dalila  (autor)
  • McCulley, Caroline  (autor)
  • Schwarz, Nele  (autor)
  • Athanasiou, Dimitra  (autor)
  • Kanuga, Naheed  (autor)
  • Novoselov, Sergey S.  (autor)
  • Lange, Clemens A.K.  (autor)
  • Ali, Robin R.  (autor)
  • Bainbridge, James W.  (autor)
  • Gias, Carlos  (autor)
  • Coffey, Peter J.  (autor)
  • Garriga Sole, Pere  (autor)
  • Cheetham, Michael E  (autor)