Journal of Molecular and Cellular Cardiology
Volume 49, Issue 4 , Pages 554-555 , October 2010

A “rough” journey to the sarcoplasmic reticulum—implications of altered calsequestrin trafficking for cardiac arrhythmia

  • Bjorn C. Knollmann

      Affiliations

    • Corresponding Author InformationCorresponding author. Oates Institute for Experimental Therapeutics, Vanderbilt University School of Medicine, 1265 Medical Research Building IV, Nashville, TN 37232-0575, USA. Tel.: +1 615 343 6493.

Received 21 June 2010 ,Accepted 23 June 2010.

References 

  1. MacLennan DH, Wong PT. Isolation of a calcium-sequestering protein from sarcoplasmic reticulum. Proc Natl Acad Sci U.S.A. 1971 Jun;68(6):1231–1235
  2. Knollmann BC. New roles of calsequestrin and triadin in cardiac muscle. J Physiol. 2009 Jul 1;587(Pt 13):3081–3087
  3. Lahooti H, Parmar KR, Wall JR. Pathogenesis of thyroid-associated ophthalmopathy: does autoimmunity against calsequestrin and collagen XIII play a role?. Clin Ophthalmol. 2010;4:417–425
  4. Park H, Wu S, Dunker AK, Kang C. Polymerization of calsequestrin. Implications for Ca2+ regulation. J Biol Chem. 2003 May 2;278(18):16176–16182
  5. Milstein ML, Houle TD, Cala SE. Calsequestrin isoforms localize to different ER subcompartments: evidence for polymer and heteropolymer-dependent localization. Exp Cell Res. 2009 Feb 1;315(3):523–534
  6. Zhang L, Kelley J, Schmeisser G, Kobayashi YM, Jones LR. Complex formation between junctin, triadin, calsequestrin, and the ryanodine receptor. Proteins of the cardiac junctional sarcoplasmic reticulum membrane. J Biol Chem. 1997 Sep 12;272(37):23389–23397
  7. Scriven DR, Dan P, Moore ED. Distribution of proteins implicated in excitation–contraction coupling in rat ventricular myocytes. Biophys J. 2000 Nov;79(5):2682–2691
  8. Jorgensen AO, Shen AC, Campbell KP. Ultrastructural localization of calsequestrin in adult rat atrial and ventricular muscle cells. J Cell Biol. 1985 Jul;101(1):257–268
  9. Chopra N, Yang T, Asghari P, Moore ED, Huke S, Akin B, et al. Ablation of triadin causes loss of cardiac Ca2+ release units, impaired excitation–contraction coupling, and cardiac arrhythmias. Proc Natl Acad Sci U.S.A. 2009 May 5;106(18):7636–7641
  10. Knollmann BC, Chopra N, Hlaing T, Akin B, Yang T, Ettensohn K, et al. Casq2 deletion causes sarcoplasmic reticulum volume increase, premature Ca2+ release, and catecholaminergic polymorphic ventricular tachycardia. J Clin Invest. 2006 Sep;116(9):2510–2520
  11. Ikemoto N, Ronjat M, Meszaros LG, Koshita M. Postulated role of calsequestrin in the regulation of calcium release from sarcoplasmic reticulum. Biochemistry. 1989 Aug 8;28(16):6764–6771
  12. Gyorke I, Hester N, Jones LR, Gyorke S. The role of calsequestrin, triadin, and junctin in conferring cardiac ryanodine receptor responsiveness to luminal calcium. Biophys J. 2004 Apr;86(4):2121–2128
  13. Jiang D, Chen W, Wang R, Zhang L, Chen SR. Loss of luminal Ca2+ activation in the cardiac ryanodine receptor is associated with ventricular fibrillation and sudden death. Proc Natl Acad Sci U.S.A. 2007 Nov 13;104(46):18309–18314
  14. Viatchenko-Karpinski S, Terentyev D, Gyorke I, Terentyeva R, Volpe P, Priori SG, et al. Abnormal calcium signaling and sudden cardiac death associated with mutation of calsequestrin. Circ Res. 2004 Mar 5;94(4):471–477
  15. Terentyev D, Nori A, Santoro M, Viatchenko-Karpinski S, Kubalova Z, Gyorke I. Abnormal interactions of calsequestrin with the ryanodine receptor calcium release channel complex linked to exercise-induced sudden cardiac death. Circ Res. 2006 Apr 6;
  16. Song L, Alcalai R, Arad M, Wolf CM, Toka O, Conner DA, et al. Calsequestrin 2 (CASQ2) mutations increase expression of calreticulin and ryanodine receptors, causing catecholaminergic polymorphic ventricular tachycardia. J Clin Invest. 2007 Jul;117(7):1814–1823
  17. Rizzi N, Liu N, Napolitano C, Nori A, Turcato F, Colombi B, et al. Unexpected structural and functional consequences of the R33Q homozygous mutation in cardiac calsequestrin: a complex arrhythmogenic cascade in a knock in mouse model. Circ Res. 2008 Aug 1;103(3):298–306
  18. Houle TD, Ram ML, Cala SE. Calsequestrin mutant D307H exhibits depressed binding to its protein targets and a depressed response to calcium. Cardiovasc Res. 2004 Nov 1;64(2):227–233
  19. Kalyanasundaram A, Bal NC, Franzini-Armstrong C, Knollmann BC, Periasamy M. The calsequestrin mutation CASQ2D307H does not affect protein stability and targeting to the junctional sarcoplasmic reticulum but compromises its dynamic regulation of calcium buffering. J Biol Chem. 2010 Jan 29;285(5):3076–3083
  20. Bal NC, Sharon A, Gupta SC, Jena N, Shaikh S, Gyorke S, et al. The catecholaminergic polymorphic ventricular tachycardia mutation R33Q disrupts the N-terminal structural motif that regulates reversible calsequestrin polymerization. J Biol Chem. 2010 May 28;285(22):17188–17196
  21. Kiarash A, Kelly CE, Phinney BS, Valdivia HH, Abrams J, Cala SE. Defective glycosylation of calsequestrin in heart failure. Cardiovasc Res. 2004 Aug 1;63(2):264–272
  22. Chopra N, Kannankeril PJ, Yang T, Hlaing T, Holinstat I, Ettensohn K, et al. Modest reductions of cardiac calsequestrin increase sarcoplasmic reticulum Ca2+ leak independent of luminal Ca2+ and trigger ventricular arrhythmias in mice. Circ Res. 2007 Sep 14;101(6):617–626

PII: S0022-2828(10)00248-8

doi: 10.1016/j.yjmcc.2010.06.011

Journal of Molecular and Cellular Cardiology
Volume 49, Issue 4 , Pages 554-555 , October 2010

Article Tools