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Selective degradation of aggregate-prone CryAB mutants by HSPB1 is mediated by ubiquitin-proteasome pathways.

Zhang H, Rajasekaran NS, Orosz A, Xiao X, Rechsteiner M, Benjamin IJ.

J Mol Cell Cardiol. 2010 Dec;49(6):918-30. doi: 10.1016/j.yjmcc.2010.09.004. Epub 2010 Sep 21.


Sumo E2 enzyme UBC9 is required for efficient protein quality control in cardiomyocytes.

Gupta MK, Gulick J, Liu R, Wang X, Molkentin JD, Robbins J.

Circ Res. 2014 Sep 26;115(8):721-9. doi: 10.1161/CIRCRESAHA.115.304760. Epub 2014 Aug 5.


COP9 signalosome controls the degradation of cytosolic misfolded proteins and protects against cardiac proteotoxicity.

Su H, Li J, Zhang H, Ma W, Wei N, Liu J, Wang X.

Circ Res. 2015 Nov 6;117(11):956-66. doi: 10.1161/CIRCRESAHA.115.306783. Epub 2015 Sep 17.


The therapeutic potential of heat shock proteins in cardiomyopathies due to mutations in cardiac structural proteins.

Rodríguez JE, Willis MS.

J Mol Cell Cardiol. 2010 Dec;49(6):904-7. doi: 10.1016/j.yjmcc.2010.09.022. Epub 2010 Oct 1. No abstract available.


Congenital cataract causing mutants of αA-crystallin/sHSP form aggregates and aggresomes degraded through ubiquitin-proteasome pathway.

Raju I, Abraham EC.

PLoS One. 2011;6(11):e28085. doi: 10.1371/journal.pone.0028085. Epub 2011 Nov 29.


Atg7 induces basal autophagy and rescues autophagic deficiency in CryABR120G cardiomyocytes.

Pattison JS, Osinska H, Robbins J.

Circ Res. 2011 Jul 8;109(2):151-60. doi: 10.1161/CIRCRESAHA.110.237339. Epub 2011 May 26.


Interruption of CryAB-amyloid oligomer formation by HSP22.

Sanbe A, Yamauchi J, Miyamoto Y, Fujiwara Y, Murabe M, Tanoue A.

J Biol Chem. 2007 Jan 5;282(1):555-63. Epub 2006 Nov 8.


Analysis of the dominant effects mediated by wild type or R120G mutant of αB-crystallin (HspB5) towards Hsp27 (HspB1).

Simon S, Dimitrova V, Gibert B, Virot S, Mounier N, Nivon M, Kretz-Remy C, Corset V, Mehlen P, Arrigo AP.

PLoS One. 2013 Aug 12;8(8):e70545. doi: 10.1371/journal.pone.0070545. eCollection 2013.


Protein kinase g positively regulates proteasome-mediated degradation of misfolded proteins.

Ranek MJ, Terpstra EJ, Li J, Kass DA, Wang X.

Circulation. 2013 Jul 23;128(4):365-76. doi: 10.1161/CIRCULATIONAHA.113.001971. Epub 2013 Jun 14.


Exercise reverses preamyloid oligomer and prolongs survival in alphaB-crystallin-based desmin-related cardiomyopathy.

Maloyan A, Gulick J, Glabe CG, Kayed R, Robbins J.

Proc Natl Acad Sci U S A. 2007 Apr 3;104(14):5995-6000. Epub 2007 Mar 26.


Ubiquitin-proteasome-mediated degradation and synthesis of MyoD is modulated by alphaB-crystallin, a small heat shock protein, during muscle differentiation.

Singh BN, Rao KS, Rao ChM.

Biochim Biophys Acta. 2010 Feb;1803(2):288-99. doi: 10.1016/j.bbamcr.2009.11.009. Epub 2009 Dec 11.


Rescue of αB Crystallin (HSPB5) Mutants Associated Protein Aggregation by Co-Expression of HSPB5 Partners.

Hussein RM, Benjamin IJ, Kampinga HH.

PLoS One. 2015 May 11;10(5):e0126761. doi: 10.1371/journal.pone.0126761. eCollection 2015.


BAG3 directly interacts with mutated alphaB-crystallin to suppress its aggregation and toxicity.

Hishiya A, Salman MN, Carra S, Kampinga HH, Takayama S.

PLoS One. 2011 Mar 15;6(3):e16828. doi: 10.1371/journal.pone.0016828.


The Human 343delT HSPB5 Chaperone Associated with Early-onset Skeletal Myopathy Causes Defects in Protein Solubility.

Mitzelfelt KA, Limphong P, Choi MJ, Kondrat FD, Lai S, Kolander KD, Kwok WM, Dai Q, Grzybowski MN, Zhang H, Taylor GM, Lui Q, Thao MT, Hudson JA, Barresi R, Bushby K, Jungbluth H, Wraige E, Geurts AM, Benesch JL, Riedel M, Christians ES, Minella AC, Benjamin IJ.

J Biol Chem. 2016 Jul 15;291(29):14939-53. doi: 10.1074/jbc.M116.730481. Epub 2016 May 19.


Selective accumulation of aggregation-prone proteasome substrates in response to proteotoxic stress.

Salomons FA, Menéndez-Benito V, Böttcher C, McCray BA, Taylor JP, Dantuma NP.

Mol Cell Biol. 2009 Apr;29(7):1774-85. doi: 10.1128/MCB.01485-08. Epub 2009 Jan 21.


Mutants of human αB-crystallin cause enhanced protein aggregation and apoptosis in mammalian cells: influence of co-expression of HspB1.

Raju I, Abraham EC.

Biochem Biophys Res Commun. 2013 Jan 4;430(1):107-12. doi: 10.1016/j.bbrc.2012.11.051. Epub 2012 Nov 27.


Molecular mechanisms of α-crystallinopathy and its therapeutic strategy.

Sanbe A.

Biol Pharm Bull. 2011;34(11):1653-8. Review.


Autophagy is an adaptive response in desmin-related cardiomyopathy.

Tannous P, Zhu H, Johnstone JL, Shelton JM, Rajasekaran NS, Benjamin IJ, Nguyen L, Gerard RD, Levine B, Rothermel BA, Hill JA.

Proc Natl Acad Sci U S A. 2008 Jul 15;105(28):9745-50. doi: 10.1073/pnas.0706802105. Epub 2008 Jul 9.


Tubulin hyperacetylation is adaptive in cardiac proteotoxicity by promoting autophagy.

McLendon PM, Ferguson BS, Osinska H, Bhuiyan MS, James J, McKinsey TA, Robbins J.

Proc Natl Acad Sci U S A. 2014 Dec 2;111(48):E5178-86. doi: 10.1073/pnas.1415589111. Epub 2014 Nov 17.

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