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The giant protein titin regulates the length of the striated muscle thick filament.

Tonino P, Kiss B, Strom J, Methawasin M, Smith JE 3rd, Kolb J, Labeit S, Granzier H.

Nat Commun. 2017 Oct 19;8(1):1041. doi: 10.1038/s41467-017-01144-9.


Small-molecule inhibition of MuRF1 attenuates skeletal muscle atrophy and dysfunction in cardiac cachexia.

Bowen TS, Adams V, Werner S, Fischer T, Vinke P, Brogger MN, Mangner N, Linke A, Sehr P, Lewis J, Labeit D, Gasch A, Labeit S.

J Cachexia Sarcopenia Muscle. 2017 Dec;8(6):939-953. doi: 10.1002/jcsm.12233. Epub 2017 Sep 8.


The Binding Sites of miR-619-5p in the mRNAs of Human and Orthologous Genes.

Atambayeva S, Niyazova R, Ivashchenko A, Pyrkova A, Pinsky I, Akimniyazova A, Labeit S.

BMC Genomics. 2017 Jun 1;18(1):428. doi: 10.1186/s12864-017-3811-6.


FoxO3a suppression and VPS34 activity are essential to anti-atrophic effects of leucine in skeletal muscle.

Baptista IL, Silvestre JG, Silva WJ, Labeit S, Moriscot AS.

Cell Tissue Res. 2017 Aug;369(2):381-394. doi: 10.1007/s00441-017-2614-z. Epub 2017 Apr 24.


Dysregulated IER3 Expression is Associated with Enhanced Apoptosis in Titin-Based Dilated Cardiomyopathy.

Zhou Q, Hahn JK, Neupane B, Aidery P, Labeit S, Gawaz M, Gramlich M.

Int J Mol Sci. 2017 Mar 29;18(4). pii: E723. doi: 10.3390/ijms18040723.


Inhibition of miR-208b improves cardiac function in titin-based dilated cardiomyopathy.

Zhou Q, Schötterl S, Backes D, Brunner E, Hahn JK, Ionesi E, Aidery P, Sticht C, Labeit S, Kandolf R, Gawaz M, Gramlich M.

Int J Cardiol. 2017 Mar 1;230:634-641. doi: 10.1016/j.ijcard.2016.12.171. Epub 2016 Dec 28.


A Novel Murine Model of Parvovirus Associated Dilated Cardiomyopathy Induced by Immunization with VP1-Unique Region of Parvovirus B19.

Bogomolovas J, Šimoliūnas E, Rinkūnaitė I, Smalinskaitė L, Podkopajev A, Bironaitė D, Weis CA, Marx A, Bukelskienė V, Gretz N, Grabauskienė V, Labeit D, Labeit S.

Biomed Res Int. 2016;2016:1627184. Epub 2016 Oct 12.


Exploration of pathomechanisms triggered by a single-nucleotide polymorphism in titin's I-band: the cardiomyopathy-linked mutation T2580I.

Bogomolovas J, Fleming JR, Anderson BR, Williams R, Lange S, Simon B, Khan MM, Rudolf R, Franke B, Bullard B, Rigden DJ, Granzier H, Labeit S, Mayans O.

Open Biol. 2016 Sep;6(9). pii: 160114.


CARP interacts with titin at a unique helical N2A sequence and at the domain Ig81 to form a structured complex.

Zhou T, Fleming JR, Franke B, Bogomolovas J, Barsukov I, Rigden DJ, Labeit S, Mayans O.

FEBS Lett. 2016 Sep;590(18):3098-110. doi: 10.1002/1873-3468.12362. Epub 2016 Sep 2.


Screening for anti-titin antibodies in patients with various paraneoplastic neurological syndromes.

Berger B, Stich O, Labeit S, Rauer S.

J Neuroimmunol. 2016 Jun 15;295-296:18-20. doi: 10.1016/j.jneuroim.2016.04.004. Epub 2016 Apr 13.


Cardiac specific titin N2B exon is a novel sensitive serological marker for cardiac injury.

Bogomolovas J, Gasch A, Bajoras V, Karčiauskaitė D, Šerpytis P, Grabauskienė V, Labeit D, Labeit S.

Int J Cardiol. 2016 Jun 1;212:232-4. doi: 10.1016/j.ijcard.2016.03.045. Epub 2016 Mar 16. No abstract available.


Titin antibodies in "seronegative" myasthenia gravis--A new role for an old antigen.

Stergiou C, Lazaridis K, Zouvelou V, Tzartos J, Mantegazza R, Antozzi C, Andreetta F, Evoli A, Deymeer F, Saruhan-Direskeneli G, Durmus H, Brenner T, Vaknin A, Berrih-Aknin S, Behin A, Sharshar T, De Baets M, Losen M, Martinez-Martinez P, Kleopa KA, Zamba-Papanicolaou E, Kyriakides T, Kostera-Pruszczyk A, Szczudlik P, Szyluk B, Lavrnic D, Basta I, Peric S, Tallaksen C, Maniaol A, Gilhus NE, Casasnovas Pons C, Pitha J, Jakubíkova M, Hanisch F, Bogomolovas J, Labeit D, Labeit S, Tzartos SJ.

J Neuroimmunol. 2016 Mar 15;292:108-15. doi: 10.1016/j.jneuroim.2016.01.018. Epub 2016 Jan 27.


Sympathetic innervation controls homeostasis of neuromuscular junctions in health and disease.

Khan MM, Lustrino D, Silveira WA, Wild F, Straka T, Issop Y, O'Connor E, Cox D, Reischl M, Marquardt T, Labeit D, Labeit S, Benoit E, Molgó J, Lochmüller H, Witzemann V, Kettelhut IC, Navegantes LC, Pozzan T, Rudolf R.

Proc Natl Acad Sci U S A. 2016 Jan 19;113(3):746-50. doi: 10.1073/pnas.1524272113. Epub 2016 Jan 5. Erratum in: Proc Natl Acad Sci U S A. 2017 Jun 19;:.


Phosphorylating Titin's Cardiac N2B Element by ERK2 or CaMKIIδ Lowers the Single Molecule and Cardiac Muscle Force.

Perkin J, Slater R, Del Favero G, Lanzicher T, Hidalgo C, Anderson B, Smith JE 3rd, Sbaizero O, Labeit S, Granzier H.

Biophys J. 2015 Dec 15;109(12):2592-601. doi: 10.1016/j.bpj.2015.11.002.


Induction of Ankrd1 in Dilated Cardiomyopathy Correlates with the Heart Failure Progression.

Bogomolovas J, Brohm K, Čelutkienė J, Balčiūnaitė G, Bironaitė D, Bukelskienė V, Daunoravičus D, Witt CC, Fielitz J, Grabauskienė V, Labeit S.

Biomed Res Int. 2015;2015:273936. doi: 10.1155/2015/273936. Epub 2015 Apr 16.


Molecular mechanisms behind progressing chronic inflammatory dilated cardiomyopathy.

Bironaite D, Daunoravicius D, Bogomolovas J, Cibiras S, Vitkus D, Zurauskas E, Zasytyte I, Rucinskas K, Labeit S, Venalis A, Grabauskiene V.

BMC Cardiovasc Disord. 2015 Mar 26;15:26. doi: 10.1186/s12872-015-0017-1.


Diaphragm muscle fiber weakness and ubiquitin-proteasome activation in critically ill patients.

Hooijman PE, Beishuizen A, Witt CC, de Waard MC, Girbes AR, Spoelstra-de Man AM, Niessen HW, Manders E, van Hees HW, van den Brom CE, Silderhuis V, Lawlor MW, Labeit S, Stienen GJ, Hartemink KJ, Paul MA, Heunks LM, Ottenheijm CA.

Am J Respir Crit Care Med. 2015 May 15;191(10):1126-38. doi: 10.1164/rccm.201412-2214OC.


Antisense-mediated exon skipping: a therapeutic strategy for titin-based dilated cardiomyopathy.

Gramlich M, Pane LS, Zhou Q, Chen Z, Murgia M, Schötterl S, Goedel A, Metzger K, Brade T, Parrotta E, Schaller M, Gerull B, Thierfelder L, Aartsma-Rus A, Labeit S, Atherton JJ, McGaughran J, Harvey RP, Sinnecker D, Mann M, Laugwitz KL, Gawaz MP, Moretti A.

EMBO Mol Med. 2015 May;7(5):562-76. doi: 10.15252/emmm.201505047.


Degeneration of neuromuscular junction in age and dystrophy.

Rudolf R, Khan MM, Labeit S, Deschenes MR.

Front Aging Neurosci. 2014 May 22;6:99. doi: 10.3389/fnagi.2014.00099. eCollection 2014. Review.


Titin kinase is an inactive pseudokinase scaffold that supports MuRF1 recruitment to the sarcomeric M-line.

Bogomolovas J, Gasch A, Simkovic F, Rigden DJ, Labeit S, Mayans O.

Open Biol. 2014 May;4(5):140041. doi: 10.1098/rsob.140041.


Molecular basis for the fold organization and sarcomeric targeting of the muscle atrogin MuRF1.

Franke B, Gasch A, Rodriguez D, Chami M, Khan MM, Rudolf R, Bibby J, Hanashima A, Bogomolovas J, von Castelmur E, Rigden DJ, Uson I, Labeit S, Mayans O.

Open Biol. 2014 Mar 26;4:130172. doi: 10.1098/rsob.130172.


Inflammation-induced acute phase response in skeletal muscle and critical illness myopathy.

Langhans C, Weber-Carstens S, Schmidt F, Hamati J, Kny M, Zhu X, Wollersheim T, Koch S, Krebs M, Schulz H, Lodka D, Saar K, Labeit S, Spies C, Hubner N, Spranger J, Spuler S, Boschmann M, Dittmar G, Butler-Browne G, Mouly V, Fielitz J.

PLoS One. 2014 Mar 20;9(3):e92048. doi: 10.1371/journal.pone.0092048. eCollection 2014.


Dynamics of myosin degradation in intensive care unit-acquired weakness during severe critical illness.

Wollersheim T, Woehlecke J, Krebs M, Hamati J, Lodka D, Luther-Schroeder A, Langhans C, Haas K, Radtke T, Kleber C, Spies C, Labeit S, Schuelke M, Spuler S, Spranger J, Weber-Carstens S, Fielitz J.

Intensive Care Med. 2014 Apr;40(4):528-38. doi: 10.1007/s00134-014-3224-9. Epub 2014 Feb 15.


Removal of immunoglobulin-like domains from titin's spring segment alters titin splicing in mouse skeletal muscle and causes myopathy.

Buck D, Smith JE 3rd, Chung CS, Ono Y, Sorimachi H, Labeit S, Granzier HL.

J Gen Physiol. 2014 Feb;143(2):215-30. doi: 10.1085/jgp.201311129.


Role of autophagy, SQSTM1, SH3GLB1, and TRIM63 in the turnover of nicotinic acetylcholine receptors.

Khan MM, Strack S, Wild F, Hanashima A, Gasch A, Brohm K, Reischl M, Carnio S, Labeit D, Sandri M, Labeit S, Rudolf R.

Autophagy. 2014 Jan;10(1):123-36. doi: 10.4161/auto.26841. Epub 2013 Nov 8.


Alterations of cAMP-dependent signaling in dystrophic skeletal muscle.

Rudolf R, Khan MM, Lustrino D, Labeit S, Kettelhut IC, Navegantes LC.

Front Physiol. 2013 Oct 17;4:290. doi: 10.3389/fphys.2013.00290. Review.


Deleting exon 55 from the nebulin gene induces severe muscle weakness in a mouse model for nemaline myopathy.

Ottenheijm CA, Buck D, de Winter JM, Ferrara C, Piroddi N, Tesi C, Jasper JR, Malik FI, Meng H, Stienen GJ, Beggs AH, Labeit S, Poggesi C, Lawlor MW, Granzier H.

Brain. 2013 Jun;136(Pt 6):1718-31. doi: 10.1093/brain/awt113. Epub 2013 May 28.


Shortening of the elastic tandem immunoglobulin segment of titin leads to diastolic dysfunction.

Chung CS, Hutchinson KR, Methawasin M, Saripalli C, Smith JE 3rd, Hidalgo CG, Luo X, Labeit S, Guo C, Granzier HL.

Circulation. 2013 Jul 2;128(1):19-28. doi: 10.1161/CIRCULATIONAHA.112.001268. Epub 2013 May 24.


Fast skeletal muscle troponin activation increases force of mouse fast skeletal muscle and ameliorates weakness due to nebulin-deficiency.

Lee EJ, De Winter JM, Buck D, Jasper JR, Malik FI, Labeit S, Ottenheijm CA, Granzier H.

PLoS One. 2013;8(2):e55861. doi: 10.1371/journal.pone.0055861. Epub 2013 Feb 20.


In vivo and in vitro investigations of heterozygous nebulin knock-out mice disclose a mild skeletal muscle phenotype.

Gineste C, De Winter JM, Kohl C, Witt CC, Giannesini B, Brohm K, Le Fur Y, Gretz N, Vilmen C, Pecchi E, Jubeau M, Cozzone PJ, Stienen GJ, Granzier H, Labeit S, Ottenheijm CA, Bendahan D, Gondin J.

Neuromuscul Disord. 2013 Apr;23(4):357-69. doi: 10.1016/j.nmd.2012.12.011. Epub 2013 Feb 1.


Single molecule force spectroscopy on titin implicates immunoglobulin domain stability as a cardiac disease mechanism.

Anderson BR, Bogomolovas J, Labeit S, Granzier H.

J Biol Chem. 2013 Feb 22;288(8):5303-15. doi: 10.1074/jbc.M112.401372. Epub 2013 Jan 6.


The multifunctional Ca(2+)/calmodulin-dependent protein kinase II delta (CaMKIIδ) phosphorylates cardiac titin's spring elements.

Hidalgo CG, Chung CS, Saripalli C, Methawasin M, Hutchinson KR, Tsaprailis G, Labeit S, Mattiazzi A, Granzier HL.

J Mol Cell Cardiol. 2013 Jan;54:90-7. doi: 10.1016/j.yjmcc.2012.11.012. Epub 2012 Dec 5.


Regulation of nicotinic acetylcholine receptor turnover by MuRF1 connects muscle activity to endo/lysosomal and atrophy pathways.

Rudolf R, Bogomolovas J, Strack S, Choi KR, Khan MM, Wagner A, Brohm K, Hanashima A, Gasch A, Labeit D, Labeit S.

Age (Dordr). 2013 Oct;35(5):1663-74. doi: 10.1007/s11357-012-9468-9. Epub 2012 Sep 6.


Identification of an N-terminal inhibitory extension as the primary mechanosensory regulator of twitchin kinase.

von Castelmur E, Strümpfer J, Franke B, Bogomolovas J, Barbieri S, Qadota H, Konarev PV, Svergun DI, Labeit S, Benian GM, Schulten K, Mayans O.

Proc Natl Acad Sci U S A. 2012 Aug 21;109(34):13608-13. doi: 10.1073/pnas.1200697109. Epub 2012 Aug 6.


The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimization.

Ottenheijm CA, Granzier H, Labeit S.

Front Physiol. 2012 Feb 27;3:37. doi: 10.3389/fphys.2012.00037. eCollection 2012.


Molecular basis for clinical heterogeneity in inherited cardiomyopathies due to myopalladin mutations.

Purevjav E, Arimura T, Augustin S, Huby AC, Takagi K, Nunoda S, Kearney DL, Taylor MD, Terasaki F, Bos JM, Ommen SR, Shibata H, Takahashi M, Itoh-Satoh M, McKenna WJ, Murphy RT, Labeit S, Yamanaka Y, Machida N, Park JE, Alexander PM, Weintraub RG, Kitaura Y, Ackerman MJ, Kimura A, Towbin JA.

Hum Mol Genet. 2012 May 1;21(9):2039-53. doi: 10.1093/hmg/dds022. Epub 2012 Jan 27.


Titin-actin interaction: PEVK-actin-based viscosity in a large animal.

Chung CS, Bogomolovas J, Gasch A, Hidalgo CG, Labeit S, Granzier HL.

J Biomed Biotechnol. 2011;2011:310791. doi: 10.1155/2011/310791. Epub 2011 Nov 15.


BAG3 in heart disease: novel clues for cardiomyocyte survival from the Z-disk?

Labeit S.

Hum Mutat. 2011 Dec;32(12):iv. doi: 10.1002/humu.21637. No abstract available.


Mechanics on myocardium deficient in the N2B region of titin: the cardiac-unique spring element improves efficiency of the cardiac cycle.

Nedrud J, Labeit S, Gotthardt M, Granzier H.

Biophys J. 2011 Sep 21;101(6):1385-92. doi: 10.1016/j.bpj.2011.06.054. Epub 2011 Sep 20.


Genetic variation in titin in arrhythmogenic right ventricular cardiomyopathy-overlap syndromes.

Taylor M, Graw S, Sinagra G, Barnes C, Slavov D, Brun F, Pinamonti B, Salcedo EE, Sauer W, Pyxaras S, Anderson B, Simon B, Bogomolovas J, Labeit S, Granzier H, Mestroni L.

Circulation. 2011 Aug 23;124(8):876-85. doi: 10.1161/CIRCULATIONAHA.110.005405. Epub 2011 Aug 1.


Nebulin, a major player in muscle health and disease.

Labeit S, Ottenheijm CA, Granzier H.

FASEB J. 2011 Mar;25(3):822-9. doi: 10.1096/fj.10-157412. Epub 2010 Nov 29. Review.


Nebulette mutations are associated with dilated cardiomyopathy and endocardial fibroelastosis.

Purevjav E, Varela J, Morgado M, Kearney DL, Li H, Taylor MD, Arimura T, Moncman CL, McKenna W, Murphy RT, Labeit S, Vatta M, Bowles NE, Kimura A, Boriek AM, Towbin JA.

J Am Coll Cardiol. 2010 Oct 26;56(18):1493-502. doi: 10.1016/j.jacc.2010.05.045.


Modulation of muscle atrophy, fatigue and MLC phosphorylation by MuRF1 as indicated by hindlimb suspension studies on MuRF1-KO mice.

Labeit S, Kohl CH, Witt CC, Labeit D, Jung J, Granzier H.

J Biomed Biotechnol. 2010;2010:693741. doi: 10.1155/2010/693741. Epub 2010 Jun 24.


Dynamic distribution of muscle-specific calpain in mice has a key role in physical-stress adaptation and is impaired in muscular dystrophy.

Ojima K, Kawabata Y, Nakao H, Nakao K, Doi N, Kitamura F, Ono Y, Hata S, Suzuki H, Kawahara H, Bogomolovas J, Witt C, Ottenheijm C, Labeit S, Granzier H, Toyama-Sorimachi N, Sorimachi M, Suzuki K, Maeda T, Abe K, Aiba A, Sorimachi H.

J Clin Invest. 2010 Aug;120(8):2672-83. doi: 10.1172/JCI40658. Epub 2010 Jul 1.


Differential splicing of the large sarcomeric protein nebulin during skeletal muscle development.

Buck D, Hudson BD, Ottenheijm CA, Labeit S, Granzier H.

J Struct Biol. 2010 May;170(2):325-33. doi: 10.1016/j.jsb.2010.02.014. Epub 2010 Feb 20.


MuRF1 is a muscle fiber-type II associated factor and together with MuRF2 regulates type-II fiber trophicity and maintenance.

Moriscot AS, Baptista IL, Bogomolovas J, Witt C, Hirner S, Granzier H, Labeit S.

J Struct Biol. 2010 May;170(2):344-53. doi: 10.1016/j.jsb.2010.02.001. Epub 2010 Feb 10.


The effects of PKCalpha phosphorylation on the extensibility of titin's PEVK element.

Anderson BR, Bogomolovas J, Labeit S, Granzier H.

J Struct Biol. 2010 May;170(2):270-7. doi: 10.1016/j.jsb.2010.02.002. Epub 2010 Feb 10.


Reduced myofibrillar connectivity and increased Z-disk width in nebulin-deficient skeletal muscle.

Tonino P, Pappas CT, Hudson BD, Labeit S, Gregorio CC, Granzier H.

J Cell Sci. 2010 Feb 1;123(Pt 3):384-91. doi: 10.1242/jcs.042234. Epub 2010 Jan 5.


Atrogin-1 and MuRF1 regulate cardiac MyBP-C levels via different mechanisms.

Mearini G, Gedicke C, Schlossarek S, Witt CC, Krämer E, Cao P, Gomes MD, Lecker SH, Labeit S, Willis MS, Eschenhagen T, Carrier L.

Cardiovasc Res. 2010 Jan 15;85(2):357-66. doi: 10.1093/cvr/cvp348. Epub 2009 Oct 22.

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