{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T18:12:43Z","timestamp":1790878363421,"version":"4.1.0"},"reference-count":60,"publisher":"Springer Science and Business Media LLC","issue":"1","funder":[{"DOI":"10.13039\/501100007873","name":"Heimer Stiftung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007873","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["G1002274, grant ID 98482"],"award-info":[{"award-number":["G1002274, grant ID 98482"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011102","name":"European Union Seventh Framework Programme","doi-asserted-by":"crossref","award":["305444 (RD-Connect) and no. 305121 (NeurOmics)"],"award-info":[{"award-number":["305444 (RD-Connect) and no. 305121 (NeurOmics)"]}],"id":[{"id":"10.13039\/100011102","id-type":"DOI","asserted-by":"crossref"}]},{"name":"German Duchenne Foundation, Bochum"},{"name":"FoRUM-Ruhr University Bochum","award":["F857R-16"],"award-info":[{"award-number":["F857R-16"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1186\/s40478-017-0474-0","type":"journal-article","created":{"date-parts":[[2017,9,15]],"date-time":"2017-09-15T12:40:00Z","timestamp":1505479200000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["Translocation of molecular chaperones to the titin springs is common in skeletal myopathy patients and affects sarcomere function"],"prefix":"10.1186","volume":"5","author":[{"given":"Andreas","family":"Unger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Beckendorf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"B\u00f6hme","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudolf","family":"Kley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion","family":"von Frieling-Salewsky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanns","family":"Lochm\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Schr\u00f6der","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter O.","family":"F\u00fcrst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Vorgerd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang A.","family":"Linke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,9,15]]},"reference":[{"key":"474_CR1","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1016\/j.cell.2014.01.056","volume":"156","author":"J Alegre-Cebollada","year":"2014","unstructured":"Alegre-Cebollada J, Kosuri P, Giganti D, Eckels E, Rivas-Pardo JA, Hamdani N, Warren CM, Solaro RJ, Linke WA, Fern\u00e1ndez JM (2014) S-glutathionylation of cryptic cysteines enhances titin elasticity by blocking protein folding. Cell 156:1235\u20131246","journal-title":"Cell"},{"key":"474_CR2","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1093\/hmg\/ddi136","volume":"14","author":"H B\u00e4r","year":"2005","unstructured":"B\u00e4r H, Fischer D, Goudeau B, Kley RA, Clemen CS, Vicart P, Herrmann H, Vorgerd M, Schr\u00f6der R (2005) Pathogenic effects of a novel heterozygous R350P desmin mutation on the assembly of desmin intermediate filaments in vivo and in vitro. Hum Mol Genet 14:1251\u20131260","journal-title":"Hum Mol Genet"},{"key":"474_CR3","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1161\/01.RES.78.5.821","volume":"78","author":"R Barbato","year":"1996","unstructured":"Barbato R, Menab\u00f2 R, Dainese P, Carafoli E, Schiaffino S, Di Lisa F (1996) Binding of cytosolic proteins to myofibrils in ischemic rat hearts. Circ Res 78:821\u2013828","journal-title":"Circ Res"},{"key":"474_CR4","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1126\/science.1066648","volume":"295","author":"JM Barral","year":"2002","unstructured":"Barral JM, Hutagalung AH, Brinker A, Hartl FU, Epstein HF (2002) Role of the myosin assembly protein UNC-45 as a molecular chaperone for myosin. Science 295:669\u2013671","journal-title":"Science"},{"key":"474_CR5","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/j.tibs.2011.11.005","volume":"37","author":"E Basha","year":"2012","unstructured":"Basha E, O'Neill H, Vierling E (2012) Small heat shock proteins and alpha-crystallins: dynamic proteins with flexible functions. Trends Biochem Sci 37:106\u2013117","journal-title":"Trends Biochem Sci"},{"key":"474_CR6","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1161\/01.RES.71.2.288","volume":"71","author":"F Bennardini","year":"1992","unstructured":"Bennardini F, Wrzosek A, Chiesi M (1992) Alpha B-crystallin in cardiac tissue. Association with actin and desmin filaments. Circ Res 71:288\u2013294","journal-title":"Circ Res"},{"key":"474_CR7","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.freeradbiomed.2014.08.012","volume":"77","author":"P Bozaykut","year":"2014","unstructured":"Bozaykut P, Ozer NK, Karademir B (2014) Regulation of protein turnover by heat shock proteins. Free Radic Biol Med 77:195\u2013209","journal-title":"Free Radic Biol Med"},{"key":"474_CR8","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1002\/prca.201400015","volume":"8","author":"H Brinkmeier","year":"2014","unstructured":"Brinkmeier H, Ohlendieck K (2014) Chaperoning heat shock proteins: proteomic analysis and relevance for normal and dystrophin-deficient muscle. Proteomics Clin Appl 8:875\u2013895","journal-title":"Proteomics Clin Appl"},{"key":"474_CR9","doi-asserted-by":"crossref","first-page":"7917","DOI":"10.1074\/jbc.M307473200","volume":"279","author":"B Bullard","year":"2004","unstructured":"Bullard B, Ferguson C, Minajeva A, Leake MC, Gautel M, Labeit D, Ding L, Labeit S, Horwitz J, Leonard KR, Linke WA (2004) Association of the chaperone alpha B-crystallin with titin in heart muscle. J Biol Chem 279:7917\u20137924","journal-title":"J Biol Chem"},{"key":"474_CR10","doi-asserted-by":"crossref","first-page":"1609","DOI":"10.1093\/hmg\/ddg173","volume":"12","author":"AT Ch\u00e1vez Zobel","year":"2003","unstructured":"Ch\u00e1vez Zobel AT, Loranger A, Marceau N, Th\u00e9riault JR, Lambert H, Landry J (2003) Distinct chaperone mechanisms can delay the formation of aggresomes by the myopathy-causing R120G alphaB-crystallin mutant. Hum Mol Genet 12:1609\u20131620","journal-title":"Hum Mol Genet"},{"key":"474_CR11","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1016\/j.bbrc.2004.09.176","volume":"324","author":"IS Chernik","year":"2004","unstructured":"Chernik IS, Panasenko OO, Li Y, Marston SB, Gusev NB (2004) pH-induced changes of the structure of small heat shock proteins with molecular mass 24\/27 kDa (HspB1). Biochem Biophys Res Commun 324:1199\u20131203","journal-title":"Biochem Biophys Res Commun"},{"key":"474_CR12","doi-asserted-by":"crossref","first-page":"7207","DOI":"10.1093\/hmg\/ddv421","volume":"24","author":"F Chevessier","year":"2015","unstructured":"Chevessier F, Schuld J, Orfanos Z et al (2015) Myofibrillar instability exacerbated by acute exercise in filaminopathy. Hum Mol Genet 24:7207\u20137220","journal-title":"Hum Mol Genet"},{"key":"474_CR13","doi-asserted-by":"publisher","first-page":"e8416","DOI":"10.1371\/journal.pone.0008416","volume":"5","author":"M Codina","year":"2010","unstructured":"Codina M, Li J, Guti\u00e9rrez J, Kao JPY, Du SJ (2010) Loss of Smyhc1 or Hsp90\u03b11 function results in different effects on myofibril organization in skeletal muscles of zebrafish embryos. PLoS One 5:e8416. doi: 10.1371\/journal.pone.0008416","journal-title":"PLoS One"},{"key":"474_CR14","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1111\/j.1469-7793.2001.0605a.x","volume":"533","author":"C Cornu","year":"2001","unstructured":"Cornu C, Goubel F, Fardeau M (2001) Muscle and joint elastic properties during elbow flexion in Duchenne muscular dystrophy. J Physiol 533:605\u2013616","journal-title":"J Physiol"},{"key":"474_CR15","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1101\/gad.177758.111","volume":"26","author":"LT Donlin","year":"2012","unstructured":"Donlin LT, Andresen C, Just S, Rudensky E, Pappas CT, Kruger M, Jacobs EY, Unger A, Zieseniss A, Dobenecker MW et al (2012) Smyd2 controls cytoplasmic lysine methylation of Hsp90 and myofilament organization. Genes Dev 26:114\u2013119","journal-title":"Genes Dev"},{"key":"474_CR16","doi-asserted-by":"crossref","first-page":"3427","DOI":"10.1111\/j.1742-4658.2006.05351.x","volume":"273","author":"S Duguez","year":"2006","unstructured":"Duguez S, Bartoli M, Richard I (2006) Calpain-3: a key regulator of the sarcomere? FEBS J 273:3427\u20133436","journal-title":"FEBS J"},{"key":"474_CR17","doi-asserted-by":"crossref","first-page":"14867","DOI":"10.1074\/jbc.274.21.14867","volume":"274","author":"M Ehrnsperger","year":"1999","unstructured":"Ehrnsperger M, Lilie H, Gaestel M, Buchner J (1999) The dynamics of Hsp25 quaternary structure. Structure and function of different oligomeric species. J Biol Chem 274:14867\u201314874","journal-title":"J Biol Chem"},{"key":"474_CR18","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1007\/s00401-002-0559-z","volume":"104","author":"DJ Fischer","year":"2002","unstructured":"Fischer DJ, Matten J, Reimann C, B\u00f6nnemann SR (2002) Expression, localization and functional divergence of alphaB-crystallin and heat shock protein 27 in core myopathies and neurogenic atrophy. Acta Neuropathol 104:297\u2013304","journal-title":"Acta Neuropathol"},{"key":"474_CR19","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1152\/japplphysiol.01026.2013","volume":"116","author":"NT Frankenberg","year":"2014","unstructured":"Frankenberg NT, Lamb GD, Overgaard K, Murphy RM, Vissing K (2014) Small heat shock proteins translocate to the cytoskeleton in human skeletal muscle following eccentric exercise independently of phosphorylation. J Appl Physiol 116:1463\u20131472","journal-title":"J Appl Physiol"},{"key":"474_CR20","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1002\/mus.10247","volume":"27","author":"J Frid\u00e9n","year":"2003","unstructured":"Frid\u00e9n J, Lieber RL (2003) Spastic muscle cells are shorter and stiffer than normal cells. Muscle Nerve 27:157\u2013164","journal-title":"Muscle Nerve"},{"key":"474_CR21","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/s00401-012-1054-9","volume":"125","author":"DO F\u00fcrst","year":"2013","unstructured":"F\u00fcrst DO, Goldfarb LG, Kley RA, Vorgerd M, Oliv\u00e9 C (2013) Filamin C related myopathies: pathology and mechanisms. Acta Neuropathol 125:33\u201346","journal-title":"Acta Neuropathol"},{"key":"474_CR22","doi-asserted-by":"crossref","first-page":"1563","DOI":"10.1083\/jcb.106.5.1563","volume":"106","author":"DO F\u00fcrst","year":"1988","unstructured":"F\u00fcrst DO, Osborn M, Nave R, Weber K (1988) The organization of titin filaments in the half-sarcomere revealed by monoclonal antibodies in immunoelectron microscopy: a map of ten nonrepetitive epitopes starting at the Z line extends close to the M line. J Cell Biol 106:1563\u20131572","journal-title":"J Cell Biol"},{"key":"474_CR23","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1161\/CIRCRESAHA.111.300105","volume":"112","author":"N Hamdani","year":"2013","unstructured":"Hamdani N, Krysiak J, Kreusser MM, Neef S, Dos Remedios CG, Maier LS, Kr\u00fcger M, Backs J, Linke WA (2013) Crucial role for Ca2+\/calmodulin-dependent protein kinase-II in regulating diastolic stress of normal and failing hearts via titin phosphorylation. Circ Res 112:664\u2013674","journal-title":"Circ Res"},{"key":"474_CR24","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1242\/dev.018150","volume":"135","author":"TA Hawkins","year":"2008","unstructured":"Hawkins TA, Haramis AP, Etard C, Prodromou C, Vaughan CK, Ashworth R, Ray S, Behra M, Holder N, Talbot WS, Pearl LH, Str\u00e4hle U, Wilson SW (2008) The ATPase-dependent chaperoning activity of Hsp90a regulates thick filament formation and integration during skeletal muscle myofibrillogenesis. Development 135:1147\u20131156","journal-title":"Development"},{"key":"474_CR25","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1161\/CIRCRESAHA.109.198465","volume":"105","author":"C Hidalgo","year":"2009","unstructured":"Hidalgo C, Hudson B, Bogomolovas J, Zhu Y, Anderson B, Greaser M, Labeit S, Granzier H (2009) PKC phosphorylation of titin\u2019s PEVK element: a novel and conserved pathway for modulating myocardial stiffness. Circ Res 105:631\u2013638","journal-title":"Circ Res"},{"key":"474_CR26","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1016\/j.bpj.2011.01.059","volume":"100","author":"T Irving","year":"2011","unstructured":"Irving T, Wu Y, Bekyarova T, Farman GP, Fukuda N, Granzier H (2011) Thick-filament strain and interfilament spacing in passive muscle: effect of titin-based passive tension. Biophys J 100:1499\u20131508","journal-title":"Biophys J"},{"key":"474_CR27","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1016\/j.nmd.2014.11.001","volume":"24","author":"JC Kaplan","year":"2014","unstructured":"Kaplan JC, Hamroun D (2014) The 2015 version of the gene table of monogenic neuromuscular disorders (nuclear genome). Neuromuscul Disord 24:1123\u20131153","journal-title":"Neuromuscul Disord"},{"key":"474_CR28","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1074\/mcp.M112.023176","volume":"12","author":"RA Kley","year":"2013","unstructured":"Kley RA, Maerkens A, Leber Y et al (2013) A combined laser microdissection and mass spectrometry approach reveals new disease relevant proteins accumulating in aggregates of filaminopathy patients. Mol Cell Proteomics 12:215\u2013227","journal-title":"Mol Cell Proteomics"},{"key":"474_CR29","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1097\/WCO.0000000000000357","volume":"29","author":"RA Kley","year":"2016","unstructured":"Kley RA, Oliv\u00e9 M, Schr\u00f6der R (2016) New aspects of myofibrillar myopathies. Curr Opin Neurol 29:628\u2013634","journal-title":"Curr Opin Neurol"},{"key":"474_CR30","doi-asserted-by":"crossref","first-page":"2642","DOI":"10.1093\/brain\/aws200","volume":"135","author":"RA Kley","year":"2012","unstructured":"Kley RA, Serdaroglu-Oflazer P, Leber Y et al (2012) Pathophysiology of protein aggregation and extended phenotyping in filaminopathy. Brain 135:2642\u20132660","journal-title":"Brain"},{"key":"474_CR31","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1083\/jcb.201306077","volume":"204","author":"S K\u00f6tter","year":"2014","unstructured":"K\u00f6tter S, Unger A, Hamdani N, Lang P, Vorgerd M, Nagel-Steger L, Linke WA (2014) Human myocytes are protected from titin aggregation-induced stiffening by small heat shock proteins. J Cell Biol 204:187\u2013202","journal-title":"J Cell Biol"},{"key":"474_CR32","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1016\/j.nmd.2017.01.001","volume":"27","author":"L Lacourpaille","year":"2017","unstructured":"Lacourpaille L, Gross R, Hug F, Gu\u00e9vel A, P\u00e9r\u00e9on Y, Magot A, Hogrel JY, Nordez A (2017) Effects of Duchenne muscular dystrophy on muscle stiffness and response to electrically-induced muscle contraction: a 12-month follow-up. Neuromuscul Disord 27:214\u2013220","journal-title":"Neuromuscul Disord"},{"key":"474_CR33","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1002\/mus.24445","volume":"51","author":"L Lacourpaille","year":"2015","unstructured":"Lacourpaille L, Hug F, Gu\u00e9vel A, P\u00e9r\u00e9on Y, Magot A, Hogrel JY, Nordez A (2015) Non-invasive assessment of muscle stiffness in patients with Duchenne muscular dystrophy. Muscle Nerve 51:284\u2013286","journal-title":"Muscle Nerve"},{"key":"474_CR34","doi-asserted-by":"crossref","first-page":"C654","DOI":"10.1152\/ajpcell.00180.2012","volume":"303","author":"NT Larkins","year":"2012","unstructured":"Larkins NT, Murphy RM, Lamb GD (2012) Influences of temperature, oxidative stress, and phosphorylation on binding of heat shock proteins in skeletal muscle fibers. Am J Physiol Cell Physiol 303:C654\u2013C665","journal-title":"Am J Physiol Cell Physiol"},{"key":"474_CR35","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1186\/1471-2377-13-144","volume":"13","author":"S Lassche","year":"2013","unstructured":"Lassche S, Ottenheijm CA, Voermans NC, Westeneng HJ, Janssen BH, van der Maarel SM, Hopman MT, Padberg GW, Stienen GJ, van Engelen BG (2013) Determining the role of sarcomeric proteins in facioscapulohumeral muscular dystrophy: a study protocol. BMC Neurol 13:144. doi: 10.1186\/1471-2377-13-144","journal-title":"BMC Neurol"},{"key":"474_CR36","doi-asserted-by":"publisher","first-page":"24492","DOI":"10.1038\/srep24492","volume":"6","author":"Y Li","year":"2016","unstructured":"Li Y, Lang P, Linke WA (2016) Titin stiffness modifies the force-generating region of muscle sarcomeres. Sci Rep 6:24492. https:\/\/doi.org\/10.1038\/srep24492","journal-title":"Sci Rep"},{"key":"474_CR37","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1006\/jmbi.1996.0441","volume":"261","author":"WA Linke","year":"1996","unstructured":"Linke WA, Ivemeyer M, Olivieri N, Kolmerer B, R\u00fcegg JC, Labeit S (1996) Towards a molecular understanding of the elasticity of titin. J Mol Biol 261:62\u201371","journal-title":"J Mol Biol"},{"key":"474_CR38","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1083\/jcb.146.3.631","volume":"146","author":"WA Linke","year":"1999","unstructured":"Linke WA, Rudy DE, Centner T, Gautel M, Witt C, Labeit S, Gregorio CC (1999) I-band titin in cardiac muscle is a three-element molecular spring and is critical for maintaining thin filament structure. J Cell Biol 146:631\u2013644","journal-title":"J Cell Biol"},{"key":"474_CR39","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1152\/physiol.00005.2010","volume":"25","author":"WA Linke","year":"2010","unstructured":"Linke WA, Kr\u00fcger M (2010) The giant protein titin as an integrator of myocyte signaling pathways. Physiology (Bethesda) 25:186\u2013198","journal-title":"Physiology (Bethesda)"},{"key":"474_CR40","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.jprot.2013.04.026","volume":"90","author":"A Maerkens","year":"2013","unstructured":"Maerkens A, Kley RA, Oliv\u00e9 M et al (2013) Differential proteomic analysis of abnormal intramyoplasmic aggregates in desminopathy. J Proteome 90:14\u201327","journal-title":"J Proteome"},{"key":"474_CR41","doi-asserted-by":"publisher","unstructured":"Maerkens A, Oliv\u00e9 M, Schreiner A, et al. (2016) New insights into the protein aggregation pathology in myotilinopathy by combined proteomic and immunolocalization analyses. Acta Neuropathol Commun 3;4:8. doi: 10.1186\/s40478-016-0280-0","DOI":"10.1186\/s40478-016-0280-0"},{"key":"474_CR42","doi-asserted-by":"crossref","first-page":"3828","DOI":"10.1021\/bi900212j","volume":"48","author":"HS Mchaourab","year":"2009","unstructured":"Mchaourab HS, Godar JA, Stewart PL (2009) Structure and mechanism of protein stability sensors: chaperone activity of small heat shock proteins. Biochemistry 48:3828\u20133837","journal-title":"Biochemistry"},{"key":"474_CR43","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/S0140-6736(12)61897-2","volume":"381","author":"E Mercuri","year":"2013","unstructured":"Mercuri E, Muntoni F (2013) Muscular dystrophies. Lancet 381:845\u2013860","journal-title":"Lancet"},{"key":"474_CR44","doi-asserted-by":"crossref","first-page":"401","DOI":"10.2170\/jjphysiol.53.401","volume":"53","author":"S Miyake","year":"2003","unstructured":"Miyake S, Ishii Y, Watari T, Huang Z, Tsuchiya T (2003) The influences of L(+)-lactate and pH on contractile performance in rabbit glycerinated skeletal muscle. Jpn J Physiol 53:401\u2013409","journal-title":"Jpn J Physiol"},{"key":"474_CR45","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1152\/physrev.00023.2010","volume":"91","author":"EV Mymrikov","year":"2011","unstructured":"Mymrikov EV, Seit-Nebi AS, Gusev NB (2011) Large potentials of small heat shock proteins. Physiol Rev 91:1123\u20131159","journal-title":"Physiol Rev"},{"key":"474_CR46","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1161\/01.CIR.0000029803.93022.93","volume":"106","author":"C Neagoe","year":"2002","unstructured":"Neagoe C, Kulke M, del Monte F, Gwathmey JK, de Tombe PP, Hajjar RJ, Linke WA (2002) Titin isoform switch in ischemic human heart disease. Circulation 106:1333\u20131341","journal-title":"Circulation"},{"key":"474_CR47","doi-asserted-by":"crossref","first-page":"3977","DOI":"10.1016\/j.febslet.2014.09.013","volume":"588","author":"P Nicholls","year":"2014","unstructured":"Nicholls P, Bujalowski PJ, Epstein HF, Boehning DF, Barral JM, Oberhauser AF (2014) Chaperone-mediated reversible inhibition of the sarcomeric myosin power stroke. FEBS Lett 588:3977\u20133981","journal-title":"FEBS Lett"},{"key":"474_CR48","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1007\/s10974-005-9023-8","volume":"26","author":"K Ojima","year":"2005","unstructured":"Ojima K, Ono Y, Hata S, Koyama S, Doi N, Sorimachi H (2005) Possible functions of p94 in connectin-mediated signaling pathways in skeletal muscle cells. J Muscle Res Cell Motil 26:409\u2013417","journal-title":"J Muscle Res Cell Motil"},{"key":"474_CR49","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1113\/jphysiol.2006.116749","volume":"577","author":"MC Olsson","year":"2006","unstructured":"Olsson MC, Kr\u00fcger M, Meyer LH, Ahnlund L, Gransberg L, Linke WA, Larsson L (2006) Fibre type-specific increase in passive muscle tension in spinal cord-injured subjects with spasticity. J Physiol 577:339\u2013352","journal-title":"J Physiol"},{"key":"474_CR50","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1152\/japplphysiol.00209.2009","volume":"107","author":"GF Paulsen","year":"2009","unstructured":"Paulsen GF, Lauritzen ML, Bayer JM, Kalhovde I, Ugelstad SG, Owe J, Bergersen HLH, Raastad T (2009) Subcellular movement and expression of HSP27, alphaB-crystallin, and HSP70 after two bouts of eccentric exercise in humans. J Appl Physiol 107:570\u2013582","journal-title":"J Appl Physiol"},{"key":"474_CR51","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1085\/jgp.200509364","volume":"126","author":"LG Prado","year":"2005","unstructured":"Prado LG, Makarenko I, Andresen C, Kr\u00fcger M, Opitz CA, Linke WA (2005) Isoform diversity of giant proteins in relation to passive and active contractile properties of rabbit skeletal muscles. J Gen Physiol 126:461\u2013480","journal-title":"J Gen Physiol"},{"key":"474_CR52","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1016\/j.celrep.2016.01.025","volume":"14","author":"JA Rivas-Pardo","year":"2016","unstructured":"Rivas-Pardo JA, Eckels EC, Popa I, Kosuri P, Linke WA, Fern\u00e1ndez JM (2016) Work Done by Titin Protein Folding Assists Muscle Contraction. Cell Rep 14:1339\u20131347","journal-title":"Cell Rep"},{"key":"474_CR53","doi-asserted-by":"publisher","first-page":"e5351","DOI":"10.1371\/journal.pone.0005351","volume":"4","author":"A Sanbe","year":"2009","unstructured":"Sanbe A, Daicho T, Mizutani R, Endo T, Miyauchi N, Yamauchi J, Tanonaka K, Glabe C, Tanoue A (2009) Protective effect of geranylgeranylacetone via enhancement of HSPB8 induction in desmin-related cardiomyopathy. PLoS One 4:e5351. https:\/\/doi.org\/10.1371\/journal.pone.0005351","journal-title":"PLoS One"},{"key":"474_CR54","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1074\/jbc.M605481200","volume":"282","author":"A Sanbe","year":"2007","unstructured":"Sanbe A, Yamauchi J, Miyamoto Y, Fujiwara Y, Murabe M, Tanoue A (2007) Interruption of CryAB-amyloid oligomer formation by HSP22. J Biol Chem 282:555\u2013563","journal-title":"J Biol Chem"},{"key":"474_CR55","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1242\/jcs.00899","volume":"117","author":"R Srikakulam","year":"2004","unstructured":"Srikakulam R, Winkelmann DA (2004) Chaperone-mediated folding and assembly of myosin in striated muscle. J Cell Sci 117:641\u2013652","journal-title":"J Cell Sci"},{"key":"474_CR56","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1016\/j.cub.2013.01.064","volume":"23","author":"A Ulbricht","year":"2013","unstructured":"Ulbricht A, Eppler FJ, Tapia VE, van der Ven PF, Hampe N, Hersch N, Vakeel P, Stadel D, Haas A, Saftig P, Behrends C, F\u00fcrst DO, Volkmer R, Hoffmann B, Kolanus W, H\u00f6hfeld J (2013) Cellular mechanotransduction relies on tension-induced and chaperone-assisted autophagy. Curr Biol 23:430\u2013435","journal-title":"Curr Biol"},{"key":"474_CR57","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1097\/WCO.0000000000000375","volume":"29","author":"J Vissing","year":"2016","unstructured":"Vissing J (2016) Limb girdle muscular dystrophies: classification, clinical spectrum and emerging therapies. Curr Opin Neurol 29:635\u2013641","journal-title":"Curr Opin Neurol"},{"key":"474_CR58","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1016\/j.bbamcr.2012.09.012","volume":"1833","author":"T Voelkel","year":"2013","unstructured":"Voelkel T, Andresen C, Unger A, Just S, Rottbauer W, Linke WA (2013) Lysine methyltransferase Smyd2 regulates Hsp90-mediated protection of the sarcomeric titin springs and cardiac function. Biochim Biophys Acta 1833:812\u2013822","journal-title":"Biochim Biophys Acta"},{"key":"474_CR59","doi-asserted-by":"crossref","first-page":"998","DOI":"10.1161\/01.RES.0000102401.77712.ED","volume":"93","author":"X Wang","year":"2003","unstructured":"Wang X, Klevitsky R, Huang W, Glasford J, Li F, Robbins J (2003) AlphaB- crystallin modulates protein aggregation of abnormal desmin. Circ Res 93:998\u20131005","journal-title":"Circ Res"},{"key":"474_CR60","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1172\/JCI71919","volume":"124","author":"L Winter","year":"2014","unstructured":"Winter L, Staszewska I, Mihailovska E, Fischer I, Goldmann WH, Schr\u00f6der R, Wiche G (2014) Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle. J Clin Invest 124:1144\u20131157","journal-title":"J Clin Invest"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-017-0474-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,10,17]],"date-time":"2020-10-17T18:58:24Z","timestamp":1602961104000},"score":1,"resource":{"primary":{"URL":"http:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-017-0474-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9,15]]},"references-count":60,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["474"],"URL":"https:\/\/doi.org\/10.1186\/s40478-017-0474-0","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,9,15]]},"article-number":"72"}}