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SCA14 mutation V138E leads to partly unfolded PKCγ associated with an exposed C-terminus, altered kinetics, phosphorylation and enhanced insolubilization.
Jezierska J, Goedhart J, Kampinga HH, Reits EA, Verbeek DS. Jezierska J, et al. Among authors: kampinga hh. J Neurochem. 2014 Mar;128(5):741-51. doi: 10.1111/jnc.12491. Epub 2013 Nov 13. J Neurochem. 2014. PMID: 24134140
RhoA Activation Sensitizes Cells to Proteotoxic Stimuli by Abrogating the HSF1-Dependent Heat Shock Response.
Meijering RA, Wiersma M, van Marion DM, Zhang D, Hoogstra-Berends F, Dijkhuis AJ, Schmidt M, Wieland T, Kampinga HH, Henning RH, Brundel BJ. Meijering RA, et al. Among authors: kampinga hh. PLoS One. 2015 Jul 20;10(7):e0133553. doi: 10.1371/journal.pone.0133553. eCollection 2015. PLoS One. 2015. PMID: 26193369 Free PMC article.
Elevated mutant dynorphin A causes Purkinje cell loss and motor dysfunction in spinocerebellar ataxia type 23.
Smeets CJ, Jezierska J, Watanabe H, Duarri A, Fokkens MR, Meijer M, Zhou Q, Yakovleva T, Boddeke E, den Dunnen W, van Deursen J, Bakalkin G, Kampinga HH, van de Sluis B, Verbeek DS. Smeets CJ, et al. Among authors: kampinga hh. Brain. 2015 Sep;138(Pt 9):2537-52. doi: 10.1093/brain/awv195. Epub 2015 Jul 12. Brain. 2015. PMID: 26169942
Mutations in potassium channel kcnd3 cause spinocerebellar ataxia type 19.
Duarri A, Jezierska J, Fokkens M, Meijer M, Schelhaas HJ, den Dunnen WF, van Dijk F, Verschuuren-Bemelmans C, Hageman G, van de Vlies P, Küsters B, van de Warrenburg BP, Kremer B, Wijmenga C, Sinke RJ, Swertz MA, Kampinga HH, Boddeke E, Verbeek DS. Duarri A, et al. Among authors: kampinga hh. Ann Neurol. 2012 Dec;72(6):870-80. doi: 10.1002/ana.23700. Ann Neurol. 2012. PMID: 23280838
Identification of the Drosophila ortholog of HSPB8: implication of HSPB8 loss of function in protein folding diseases.
Carra S, Boncoraglio A, Kanon B, Brunsting JF, Minoia M, Rana A, Vos MJ, Seidel K, Sibon OC, Kampinga HH. Carra S, et al. Among authors: kampinga hh. J Biol Chem. 2010 Nov 26;285(48):37811-22. doi: 10.1074/jbc.M110.127498. Epub 2010 Sep 21. J Biol Chem. 2010. PMID: 20858900 Free PMC article.
Spinocerebellar ataxia type 19/22 mutations alter heterocomplex Kv4.3 channel function and gating in a dominant manner.
Duarri A, Lin MC, Fokkens MR, Meijer M, Smeets CJ, Nibbeling EA, Boddeke E, Sinke RJ, Kampinga HH, Papazian DM, Verbeek DS. Duarri A, et al. Among authors: kampinga hh. Cell Mol Life Sci. 2015 Sep;72(17):3387-99. doi: 10.1007/s00018-015-1894-2. Epub 2015 Apr 9. Cell Mol Life Sci. 2015. PMID: 25854634 Free PMC article.
Overexpression of the cochaperone CHIP enhances Hsp70-dependent folding activity in mammalian cells.
Kampinga HH, Kanon B, Salomons FA, Kabakov AE, Patterson C. Kampinga HH, et al. Mol Cell Biol. 2003 Jul;23(14):4948-58. doi: 10.1128/mcb.23.14.4948-4958.2003. Mol Cell Biol. 2003. PMID: 12832480 Free PMC article.
HspB8 participates in protein quality control by a non-chaperone-like mechanism that requires eIF2{alpha} phosphorylation.
Carra S, Brunsting JF, Lambert H, Landry J, Kampinga HH. Carra S, et al. Among authors: kampinga hh. J Biol Chem. 2009 Feb 27;284(9):5523-32. doi: 10.1074/jbc.M807440200. Epub 2008 Dec 29. J Biol Chem. 2009. PMID: 19114712
Comparison of intra-organellar chaperone capacity for dealing with stress-induced protein unfolding.
Hageman J, Vos MJ, van Waarde MA, Kampinga HH. Hageman J, et al. Among authors: kampinga hh. J Biol Chem. 2007 Nov 23;282(47):34334-45. doi: 10.1074/jbc.M703876200. Epub 2007 Sep 17. J Biol Chem. 2007. PMID: 17875648
Grp/DChk1 is required for G2-M checkpoint activation in Drosophila S2 cells, whereas Dmnk/DChk2 is dispensable.
de Vries HI, Uyetake L, Lemstra W, Brunsting JF, Su TT, Kampinga HH, Sibon OC. de Vries HI, et al. Among authors: kampinga hh. J Cell Sci. 2005 May 1;118(Pt 9):1833-42. doi: 10.1242/jcs.02309. J Cell Sci. 2005. PMID: 15860729 Free PMC article.
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