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Sarcomeric myopathies associated with tremor: new insights and perspectives.

Stavusis J, Geist J, Kontrogianni-Konstantopoulos A.

J Muscle Res Cell Motil. 2019 Oct 16. doi: 10.1007/s10974-019-09559-1. [Epub ahead of print]


Heterozygous variants in MYBPC1 are associated with an expanded neuromuscular phenotype beyond arthrogryposis.

Shashi V, Geist J, Lee Y, Yoo Y, Shin U, Schoch K, Sullivan J, Stong N, Smith E, Jasien J, Kranz P; Undiagnosed Diseases Network, Lee Y, Shin YB, Wright NT, Choi M, Kontrogianni-Konstantopoulos A.

Hum Mutat. 2019 Aug;40(8):1115-1126. doi: 10.1002/humu.23760. Epub 2019 May 5.


A microfluidic assay for the quantification of the metastatic propensity of breast cancer specimens.

Yankaskas CL, Thompson KN, Paul CD, Vitolo MI, Mistriotis P, Mahendra A, Bajpai VK, Shea DJ, Manto KM, Chai AC, Varadarajan N, Kontrogianni-Konstantopoulos A, Martin SS, Konstantopoulos K.

Nat Biomed Eng. 2019 Jun;3(6):452-465. doi: 10.1038/s41551-019-0400-9. Epub 2019 May 6.


Novel mutations in MYBPC1 are associated with myogenic tremor and mild myopathy.

Stavusis J, Lace B, Schäfer J, Geist J, Inashkina I, Kidere D, Pajusalu S, Wright NT, Saak A, Weinhold M, Haubenberger D, Jackson S, Kontrogianni-Konstantopoulos A, Bönnemann CG.

Ann Neurol. 2019 Jul;86(1):129-142. doi: 10.1002/ana.25494. Epub 2019 May 17.


Regulation of myofilament force and loaded shortening by skeletal myosin binding protein C.

Robinett JC, Hanft LM, Geist J, Kontrogianni-Konstantopoulos A, McDonald KS.

J Gen Physiol. 2019 May 6;151(5):645-659. doi: 10.1085/jgp.201812200. Epub 2019 Jan 31.


Unraveling obscurins in heart disease.

Grogan A, Kontrogianni-Konstantopoulos A.

Pflugers Arch. 2019 May;471(5):735-743. doi: 10.1007/s00424-018-2191-3. Epub 2018 Aug 11. Review.


Structure before function: myosin binding protein-C slow is a structural protein with regulatory properties.

Geist J, Ward CW, Kontrogianni-Konstantopoulos A.

FASEB J. 2018 Jun 6:fj201800624R. doi: 10.1096/fj.201800624R. [Epub ahead of print]


Corrigendum: The Phosphorylation Profile of Myosin Binding Protein-C Slow is Dynamically Regulated in Slow-Twitch Muscles in Health and Disease.

Ackermann MA, Kerr JP, King B, Ward CW, Kontrogianni-Konstantopoulos A.

Sci Rep. 2018 May 11;8:46969. doi: 10.1038/srep46969.


Thick Filament Protein Network, Functions, and Disease Association.

Wang L, Geist J, Grogan A, Hu LR, Kontrogianni-Konstantopoulos A.

Compr Physiol. 2018 Mar 13;8(2):631-709. doi: 10.1002/cphy.c170023. Review.


Correction: Obscurins: Goliaths and Davids Take over Non-Muscle Tissues.

Ackermann MA, Shriver M, Perry NA, Hu LR, Kontrogianni-Konstantopoulos A.

PLoS One. 2018 Jan 3;13(1):e0190842. doi: 10.1371/journal.pone.0190842. eCollection 2018.


Loss of giant obscurins alters breast epithelial cell mechanosensing of matrix stiffness.

Stroka KM, Wong BS, Shriver M, Phillip JM, Wirtz D, Kontrogianni-Konstantopoulos A, Konstantopoulos K.

Oncotarget. 2016 Aug 1;8(33):54004-54020. doi: 10.18632/oncotarget.10997. eCollection 2017 Aug 15.


Novel obscurins mediate cardiomyocyte adhesion and size via the PI3K/AKT/mTOR signaling pathway.

Ackermann MA, King B, Lieberman NAP, Bobbili PJ, Rudloff M, Berndsen CE, Wright NT, Hecker PA, Kontrogianni-Konstantopoulos A.

J Mol Cell Cardiol. 2017 Oct;111:27-39. doi: 10.1016/j.yjmcc.2017.08.004. Epub 2017 Aug 4.


Deregulated Ca2+ cycling underlies the development of arrhythmia and heart disease due to mutant obscurin.

Hu LR, Ackermann MA, Hecker PA, Prosser BL, King B, O'Connell KA, Grogan A, Meyer LC, Berndsen CE, Wright NT, Jonathan Lederer W, Kontrogianni-Konstantopoulos A.

Sci Adv. 2017 Jun 7;3(6):e1603081. doi: 10.1126/sciadv.1603081. eCollection 2017 Jun.


MYBPC1, an Emerging Myopathic Gene: What We Know and What We Need to Learn.

Geist J, Kontrogianni-Konstantopoulos A.

Front Physiol. 2016 Sep 14;7:410. doi: 10.3389/fphys.2016.00410. eCollection 2016. Review.


Giant obscurins regulate the PI3K cascade in breast epithelial cells via direct binding to the PI3K/p85 regulatory subunit.

Shriver M, Marimuthu S, Paul C, Geist J, Seale T, Konstantopoulos K, Kontrogianni-Konstantopoulos A.

Oncotarget. 2016 Jul 19;7(29):45414-45428. doi: 10.18632/oncotarget.9985.


Myosin Binding Protein-C Slow Phosphorylation is Altered in Duchenne Dystrophy and Arthrogryposis Myopathy in Fast-Twitch Skeletal Muscles.

Ackermann MA, Ward CW, Gurnett C, Kontrogianni-Konstantopoulos A.

Sci Rep. 2015 Aug 19;5:13235. doi: 10.1038/srep13235.


The Phosphorylation Profile of Myosin Binding Protein-C Slow is Dynamically Regulated in Slow-Twitch Muscles in Health and Disease.

Ackermann MA, Kerr JP, King B, W Ward C, Kontrogianni-Konstantopoulos A.

Sci Rep. 2015 Aug 19;5:12637. doi: 10.1038/srep12637. Erratum in: Sci Rep. 2018 May 11;8:46969.


The sarcomeric M-region: a molecular command center for diverse cellular processes.

Hu LY, Ackermann MA, Kontrogianni-Konstantopoulos A.

Biomed Res Int. 2015;2015:714197. doi: 10.1155/2015/714197. Epub 2015 Apr 15. Review.


Fluid shear promotes chondrosarcoma cell invasion by activating matrix metalloproteinase 12 via IGF-2 and VEGF signaling pathways.

Wang P, Chen SH, Hung WC, Paul C, Zhu F, Guan PP, Huso DL, Kontrogianni-Konstantopoulos A, Konstantopoulos K.

Oncogene. 2015 Aug 27;34(35):4558-69. doi: 10.1038/onc.2014.397. Epub 2014 Dec 1.


Loss of giant obscurins from breast epithelium promotes epithelial-to-mesenchymal transition, tumorigenicity and metastasis.

Shriver M, Stroka KM, Vitolo MI, Martin S, Huso DL, Konstantopoulos K, Kontrogianni-Konstantopoulos A.

Oncogene. 2015 Aug 6;34(32):4248-59. doi: 10.1038/onc.2014.358. Epub 2014 Nov 10.


Loss of the obscurin-RhoGEF downregulates RhoA signaling and increases microtentacle formation and attachment of breast epithelial cells.

Perry NA, Vitolo MI, Martin SS, Kontrogianni-Konstantopoulos A.

Oncotarget. 2014 Sep 30;5(18):8558-68.


Obscurins: Goliaths and Davids take over non-muscle tissues.

Ackermann MA, Shriver M, Perry NA, Hu LY, Kontrogianni-Konstantopoulos A.

PLoS One. 2014 Feb 6;9(2):e88162. doi: 10.1371/journal.pone.0088162. eCollection 2014. Erratum in: PLoS One. 2018 Jan 3;13(1):e0190842.


Myosin binding protein-C slow: a multifaceted family of proteins with a complex expression profile in fast and slow twitch skeletal muscles.

Ackermann MA, Kontrogianni-Konstantopoulos A.

Front Physiol. 2013 Dec 25;4:391. doi: 10.3389/fphys.2013.00391. eCollection 2013.


Competition through dimerization between antiapoptotic and proapoptotic HS-1-associated protein X-1 (Hax-1).

Koontz J, Kontrogianni-Konstantopoulos A.

J Biol Chem. 2014 Feb 7;289(6):3468-77. doi: 10.1074/jbc.M113.536151. Epub 2013 Dec 17.


Loss of actomyosin regulation in distal arthrogryposis myopathy due to mutant myosin binding protein-C slow.

Ackermann MA, Patel PD, Valenti J, Takagi Y, Homsher E, Sellers JR, Kontrogianni-Konstantopoulos A.

FASEB J. 2013 Aug;27(8):3217-28. doi: 10.1096/fj.13-228882. Epub 2013 May 8. Erratum in: FASEB J. 2018 Mar;32(3):1729-1730.


Obscurins: unassuming giants enter the spotlight.

Perry NA, Ackermann MA, Shriver M, Hu LY, Kontrogianni-Konstantopoulos A.

IUBMB Life. 2013 Jun;65(6):479-86. doi: 10.1002/iub.1157. Epub 2013 Mar 20. Review.


The kinase domains of obscurin interact with intercellular adhesion proteins.

Hu LY, Kontrogianni-Konstantopoulos A.

FASEB J. 2013 May;27(5):2001-12. doi: 10.1096/fj.12-221317. Epub 2013 Feb 7.


Advances in Muscle Physiology and Pathophysiology 2011.

Kontrogianni-Konstantopoulos A, Benian G, Granzier H.

J Biomed Biotechnol. 2012;2012:930836. doi: 10.1155/2012/930836. Epub 2012 Jan 15. No abstract available.


Loss of giant obscurins promotes breast epithelial cell survival through apoptotic resistance.

Perry NA, Shriver M, Mameza MG, Grabias B, Balzer E, Kontrogianni-Konstantopoulos A.

FASEB J. 2012 Jul;26(7):2764-75. doi: 10.1096/fj.12-205419. Epub 2012 Mar 21. Erratum in: FASEB J. 2012 Sep;26(9):3931.


Integrity of the network sarcoplasmic reticulum in skeletal muscle requires small ankyrin 1.

Ackermann MA, Ziman AP, Strong J, Zhang Y, Hartford AK, Ward CW, Randall WR, Kontrogianni-Konstantopoulos A, Bloch RJ.

J Cell Sci. 2011 Nov 1;124(Pt 21):3619-30. doi: 10.1242/jcs.085159. Epub 2011 Nov 1.


Myosin binding protein-C: a regulator of actomyosin interaction in striated muscle.

Ackermann MA, Kontrogianni-Konstantopoulos A.

J Biomed Biotechnol. 2011;2011:636403. doi: 10.1155/2011/636403. Epub 2011 Oct 16. Review.


Myosin binding protein-C slow is a novel substrate for protein kinase A (PKA) and C (PKC) in skeletal muscle.

Ackermann MA, Kontrogianni-Konstantopoulos A.

J Proteome Res. 2011 Oct 7;10(10):4547-55. doi: 10.1021/pr200355w. Epub 2011 Sep 22.


Resisting resistin; it's good for the heart.

Kontrogianni-Konstantopoulos A.

J Mol Cell Cardiol. 2011 Aug;51(2):141-3. doi: 10.1016/j.yjmcc.2011.05.011. Epub 2011 May 24. No abstract available.


Electrostatic interactions mediate binding of obscurin to small ankyrin 1: biochemical and molecular modeling studies.

Busby B, Oashi T, Willis CD, Ackermann MA, Kontrogianni-Konstantopoulos A, Mackerell AD Jr, Bloch RJ.

J Mol Biol. 2011 Apr 29;408(2):321-34. doi: 10.1016/j.jmb.2011.01.053. Epub 2011 Feb 17.


HAX-1: a family of apoptotic regulators in health and disease.

Yap SV, Koontz JM, Kontrogianni-Konstantopoulos A.

J Cell Physiol. 2011 Nov;226(11):2752-61. doi: 10.1002/jcp.22638. Review.


Characterization and comparison of two binding sites on obscurin for small ankyrin 1.

Busby B, Willis CD, Ackermann MA, Kontrogianni-Konstantopoulos A, Bloch RJ.

Biochemistry. 2010 Nov 23;49(46):9948-56. doi: 10.1021/bi101165p. Epub 2010 Nov 1.


Advances in muscle physiology and pathophysiology.

Kontrogianni-Konstantopoulos A, Benian G, Granzier H.

J Biomed Biotechnol. 2010;2010. pii: 780417. doi: 10.1155/2010/780417. Epub 2010 Jul 27. No abstract available.


Myosin binding protein-C slow: an intricate subfamily of proteins.

Ackermann MA, Kontrogianni-Konstantopoulos A.

J Biomed Biotechnol. 2010;2010:652065. doi: 10.1155/2010/652065. Epub 2010 Apr 8. Review.


Prostaglandin (PG)D(2) and 15-deoxy-Delta(12,14)-PGJ(2), but not PGE(2), mediate shear-induced chondrocyte apoptosis via protein kinase A-dependent regulation of polo-like kinases.

Zhu F, Wang P, Kontrogianni-Konstantopoulos A, Konstantopoulos K.

Cell Death Differ. 2010 Aug;17(8):1325-34. doi: 10.1038/cdd.2010.13. Epub 2010 Feb 12.


The anti-apoptotic protein HAX-1 is a regulator of cardiac function.

Zhao W, Waggoner JR, Zhang ZG, Lam CK, Han P, Qian J, Schroder PM, Mitton B, Kontrogianni-Konstantopoulos A, Robia SL, Kranias EG.

Proc Natl Acad Sci U S A. 2009 Dec 8;106(49):20776-81. doi: 10.1073/pnas.0906998106. Epub 2009 Nov 17.


HAX-1: a multifaceted antiapoptotic protein localizing in the mitochondria and the sarcoplasmic reticulum of striated muscle cells.

Yap SV, Vafiadaki E, Strong J, Kontrogianni-Konstantopoulos A.

J Mol Cell Cardiol. 2010 Jun;48(6):1266-79. doi: 10.1016/j.yjmcc.2009.10.028. Epub 2009 Nov 11.


Muscle giants: molecular scaffolds in sarcomerogenesis.

Kontrogianni-Konstantopoulos A, Ackermann MA, Bowman AL, Yap SV, Bloch RJ.

Physiol Rev. 2009 Oct;89(4):1217-67. doi: 10.1152/physrev.00017.2009. Review.


Obscurin interacts with a novel isoform of MyBP-C slow at the periphery of the sarcomeric M-band and regulates thick filament assembly.

Ackermann MA, Hu LY, Bowman AL, Bloch RJ, Kontrogianni-Konstantopoulos A.

Mol Biol Cell. 2009 Jun;20(12):2963-78. doi: 10.1091/mbc.E08-12-1251. Epub 2009 Apr 29.


The anti-apoptotic protein HAX-1 interacts with SERCA2 and regulates its protein levels to promote cell survival.

Vafiadaki E, Arvanitis DA, Pagakis SN, Papalouka V, Sanoudou D, Kontrogianni-Konstantopoulos A, Kranias EG.

Mol Biol Cell. 2009 Jan;20(1):306-18. doi: 10.1091/mbc.E08-06-0587. Epub 2008 Oct 29.


The Ser96Ala variant in histidine-rich calcium-binding protein is associated with life-threatening ventricular arrhythmias in idiopathic dilated cardiomyopathy.

Arvanitis DA, Sanoudou D, Kolokathis F, Vafiadaki E, Papalouka V, Kontrogianni-Konstantopoulos A, Theodorakis GN, Paraskevaidis IA, Adamopoulos S, Dorn GW 2nd, Kremastinos DT, Kranias EG.

Eur Heart J. 2008 Oct;29(20):2514-25. doi: 10.1093/eurheartj/ehn328. Epub 2008 Jul 9.


The rho-guanine nucleotide exchange factor domain of obscurin regulates assembly of titin at the Z-disk through interactions with Ran binding protein 9.

Bowman AL, Catino DH, Strong JC, Randall WR, Kontrogianni-Konstantopoulos A, Bloch RJ.

Mol Biol Cell. 2008 Sep;19(9):3782-92. doi: 10.1091/mbc.E08-03-0237. Epub 2008 Jun 25.


Mapping the binding site on small ankyrin 1 for obscurin.

Borzok MA, Catino DH, Nicholson JD, Kontrogianni-Konstantopoulos A, Bloch RJ.

J Biol Chem. 2007 Nov 2;282(44):32384-96. Epub 2007 Aug 25.


Histidine-rich Ca-binding protein interacts with sarcoplasmic reticulum Ca-ATPase.

Arvanitis DA, Vafiadaki E, Fan GC, Mitton BA, Gregory KN, Del Monte F, Kontrogianni-Konstantopoulos A, Sanoudou D, Kranias EG.

Am J Physiol Heart Circ Physiol. 2007 Sep;293(3):H1581-9. Epub 2007 May 25.


Different obscurin isoforms localize to distinct sites at sarcomeres.

Bowman AL, Kontrogianni-Konstantopoulos A, Hirsch SS, Geisler SB, Gonzalez-Serratos H, Russell MW, Bloch RJ.

FEBS Lett. 2007 Apr 17;581(8):1549-54. Epub 2007 Mar 15.


Phospholamban interacts with HAX-1, a mitochondrial protein with anti-apoptotic function.

Vafiadaki E, Sanoudou D, Arvanitis DA, Catino DH, Kranias EG, Kontrogianni-Konstantopoulos A.

J Mol Biol. 2007 Mar 16;367(1):65-79. Epub 2006 Oct 21.


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