Stephan Lange

Title(s)Associate Adjunct Professor, Medicine
SchoolHealth Sciences
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    Collapse Biography 
    Collapse Awards and Honors
    International Society for Heart Research2014ISHR Young Investigator Award (Winner Senior Group)
    NIH2011NIH K99 Pathway to Independence Award
    2010Travel Award of the European Muscle Conference (EMC)
    2009University of San Diego Cardiovascular Scholarship Award
    2007Travel Award of the British Society of Cell Biology
    2007Travel Award of the Swiss Society of Cell Biology
    2007Poster Award of the European Muscle Conference (EMC)
    Swiss Federal Institute of Technology2006Medal of the Swiss Federal Institute of Technology
    2005Travel Award of the Weinstein Conference on Cardiovascular Development
    2005Travel Award of the British Society of Cell Biology

    Collapse Overview 
    Collapse Overview
    Dr. Lange's laboratory investigates the development and function of heart and skeletal muscles. The research focus is centered on the identification of molecular origins and pathways that lead to the development of cardiomyopathies and muscular dystrophies. We are particularly interested how mutations in sarcomeric and muscle associated proteins lead to the development of these diseases.

    Collapse Research 
    Collapse Research Activities and Funding
    Signaling Pathways in Dilated Cardiomyopathy
    NIH/NHLBI R01HL128457Aug 1, 2015 - Jul 31, 2020
    Role: Principal Investigator
    Cullin mediated protein turnover in skeletal and cardiac muscles
    NIH/NHLBI R00HL107744Apr 1, 2011 - Mar 31, 2016
    Role: Principal Investigator
    Cullin mediated protein turnover in skeletal and cardiac muscles
    NIH/NHLBI K99HL107744Apr 1, 2011 - Mar 31, 2013
    Role: Principal Investigator

    Collapse ORNG Applications 
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    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
    List All   |   Timeline
    1. Blondelle J, Marrocco V, Clark M, Desmond P, Myers S, Nguyen J, Wright M, Bremner S, Pierantozzi E, Ward S, Estève E, Sorrentino V, Ghassemian M, Lange S. Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism. Commun Biol. 2019; 2:178. PMID: 31098411.
      View in: PubMed
    2. Blondelle J, Tallapaka K, Seto JT, Ghassemian M, Clark M, Laitila JM, Bournazos A, Singer JD, Lange S. Cullin-3 dependent deregulation of ACTN1 represents a new pathogenic mechanism in nemaline myopathy. JCI Insight. 2019 Apr 16; 5. PMID: 30990797.
      View in: PubMed
    3. Marrocco V, Bogomolovas J, Ehler E, Dos Remedios CG, Yu J, Gao C, Lange S. PKC and PKN in heart disease. J Mol Cell Cardiol. 2019 Mar; 128:212-226. PMID: 30742812.
      View in: PubMed
    4. Lange S, Pinotsis N, Agarkova I, Ehler E. The M-band: The underestimated part of the sarcomere. Biochim Biophys Acta Mol Cell Res. 2019 Feb 07. PMID: 30738787.
      View in: PubMed
    5. Ehsan M, Kelly M, Hooper C, Yavari A, Beglov J, Bellahcene M, Ghataorhe K, Poloni G, Goel A, Kyriakou T, Fleischanderl K, Ehler E, Makeyev E, Lange S, Ashrafian H, Redwood C, Davies B, Watkins H, Gehmlich K. Mutant Muscle LIM Protein C58G causes cardiomyopathy through protein depletion. J Mol Cell Cardiol. 2018 Aug; 121:287-296. PMID: 30048712.
      View in: PubMed
    6. Fang X, Bogomolovas J, Wu T, Zhang W, Liu C, Veevers J, Stroud MJ, Zhang Z, Ma X, Mu Y, Lao DH, Dalton ND, Gu Y, Wang C, Wang M, Liang Y, Lange S, Ouyang K, Peterson KL, Evans SM, Chen J. Loss-of-function mutations in co-chaperone BAG3 destabilize small HSPs and cause cardiomyopathy. J Clin Invest. 2017 Aug 01; 127(8):3189-3200. PMID: 28737513.
      View in: PubMed
    7. Blondelle J, Shapiro P, Domenighetti AA, Lange S. Cullin E3 Ligase Activity Is Required for Myoblast Differentiation. J Mol Biol. 2017 04 07; 429(7):1045-1066. PMID: 28238764.
      View in: PubMed
    8. 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. Exploration of pathomechanisms triggered by a single-nucleotide polymorphism in titin's I-band: the cardiomyopathy-linked mutation T2580I. Open Biol. 2016 Sep; 6(9). PMID: 27683155.
      View in: PubMed
    9. Lange S, Gehmlich K, Lun AS, Blondelle J, Hooper C, Dalton ND, Alvarez EA, Zhang X, Bang ML, Abassi YA, Dos Remedios CG, Peterson KL, Chen J, Ehler E. MLP and CARP are linked to chronic PKCa signalling in dilated cardiomyopathy. Nat Commun. 2016 06 29; 7:12120. PMID: 27353086.
      View in: PubMed
    10. Mu Y, Jing R, Peter AK, Lange S, Lin L, Zhang J, Ouyang K, Fang X, Veevers J, Zhou X, Evans SM, Cheng H, Chen J. Cypher and Enigma homolog protein are essential for cardiac development and embryonic survival. J Am Heart Assoc. 2015 May 05; 4(5). PMID: 25944877; PMCID: PMC4599425.
    11. Blondelle J, Lange S, Greenberg BH, Cowling RT. Cathepsins in heart disease-chewing on the heartache? Am J Physiol Heart Circ Physiol. 2015 May 01; 308(9):H974-6. PMID: 25747750; PMCID: PMC4578146.
    12. Banerjee I, Carrion K, Serrano R, Dyo J, Sasik R, Lund S, Willems E, Aceves S, Meili R, Mercola M, Chen J, Zambon A, Hardiman G, Doherty TA, Lange S, del Álamo JC, Nigam V. Cyclic stretch of embryonic cardiomyocytes increases proliferation, growth, and expression while repressing Tgf-ß signaling. J Mol Cell Cardiol. 2015 Feb; 79:133-44. PMID: 25446186.
      View in: PubMed
    13. Lun AS, Chen J, Lange S. Probing muscle ankyrin-repeat protein (MARP) structure and function. Anat Rec (Hoboken). 2014 Sep; 297(9):1615-29. PMID: 25125175; PMCID: PMC4135402.
    14. Lange S, Edström L, Udd B, Gautel M. Reply: Hereditary myopathy with early respiratory failure is caused by mutations in the titin FN3 119 domain. Brain. 2014 Jun; 137(Pt 6):e279. PMID: 24569025; PMCID: PMC4032095.
    15. Ehler E, Moore-Morris T, Lange S. Isolation and culture of neonatal mouse cardiomyocytes. J Vis Exp. 2013 Sep 06; (79). PMID: 24056408; PMCID: PMC3857885.
    16. Lin C, Guo X, Lange S, Liu J, Ouyang K, Yin X, Jiang L, Cai Y, Mu Y, Sheikh F, Ye S, Chen J, Ke Y, Cheng H. Cypher/ZASP is a novel A-kinase anchoring protein. J Biol Chem. 2013 Oct 11; 288(41):29403-13. PMID: 23996002; PMCID: PMC3795241.
    17. Lyon RC, Lange S, Sheikh F. Breaking down protein degradation mechanisms in cardiac muscle. Trends Mol Med. 2013 Apr; 19(4):239-49. PMID: 23453282; PMCID: PMC3622835.
    18. Randazzo D, Giacomello E, Lorenzini S, Rossi D, Pierantozzi E, Blaauw B, Reggiani C, Lange S, Peter AK, Chen J, Sorrentino V. Obscurin is required for ankyrinB-dependent dystrophin localization and sarcolemma integrity. J Cell Biol. 2013 Feb 18; 200(4):523-36. PMID: 23420875; PMCID: PMC3575540.
    19. Raskin A, Lange S, Banares K, Lyon RC, Zieseniss A, Lee LK, Yamazaki KG, Granzier HL, Gregorio CC, McCulloch AD, Omens JH, Sheikh F. A novel mechanism involving four-and-a-half LIM domain protein-1 and extracellular signal-regulated kinase-2 regulates titin phosphorylation and mechanics. J Biol Chem. 2012 Aug 24; 287(35):29273-84. PMID: 22778266; PMCID: PMC3436149.
    20. Lange S, Perera S, Teh P, Chen J. Obscurin and KCTD6 regulate cullin-dependent small ankyrin-1 (sAnk1.5) protein turnover. Mol Biol Cell. 2012 Jul; 23(13):2490-504. PMID: 22573887; PMCID: PMC3386213.
    21. Banerjee I, Zhang J, Moore-Morris T, Lange S, Shen T, Dalton ND, Gu Y, Peterson KL, Evans SM, Chen J. Thymosin beta 4 is dispensable for murine cardiac development and function. Circ Res. 2012 Feb 03; 110(3):456-64. PMID: 22158707; PMCID: PMC3739283.
    22. Iskratsch T, Lange S, Dwyer J, Kho AL, dos Remedios C, Ehler E. Formin follows function: a muscle-specific isoform of FHOD3 is regulated by CK2 phosphorylation and promotes myofibril maintenance. J Cell Biol. 2010 Dec 13; 191(6):1159-72. PMID: 21149568; PMCID: PMC3002041.
    23. DeQuach JA, Mezzano V, Miglani A, Lange S, Keller GM, Sheikh F, Christman KL. Simple and high yielding method for preparing tissue specific extracellular matrix coatings for cell culture. PLoS One. 2010 Sep 27; 5(9):e13039. PMID: 20885963; PMCID: PMC2946408.
    24. Zhang J, Felder A, Liu Y, Guo LT, Lange S, Dalton ND, Gu Y, Peterson KL, Mizisin AP, Shelton GD, Lieber RL, Chen J. Nesprin 1 is critical for nuclear positioning and anchorage. Hum Mol Genet. 2010 Jan 15; 19(2):329-41. PMID: 19864491; PMCID: PMC2796894.
    25. Lange S, Ouyang K, Meyer G, Cui L, Cheng H, Lieber RL, Chen J. Obscurin determines the architecture of the longitudinal sarcoplasmic reticulum. J Cell Sci. 2009 Aug 01; 122(Pt 15):2640-50. PMID: 19584095; PMCID: PMC2909316.
    26. Sheikh F, Raskin A, Chu PH, Lange S, Domenighetti AA, Zheng M, Liang X, Zhang T, Yajima T, Gu Y, Dalton ND, Mahata SK, Dorn GW, Brown JH, Heller-Brown J, Peterson KL, Omens JH, McCulloch AD, Chen J. An FHL1-containing complex within the cardiomyocyte sarcomere mediates hypertrophic biomechanical stress responses in mice. J Clin Invest. 2008 Dec; 118(12):3870-80. PMID: 19033658; PMCID: PMC2575833.
    27. Fukuzawa A, Lange S, Holt M, Vihola A, Carmignac V, Ferreiro A, Udd B, Gautel M. Interactions with titin and myomesin target obscurin and obscurin-like 1 to the M-band: implications for hereditary myopathies. J Cell Sci. 2008 Jun 01; 121(11):1841-51. PMID: 18477606.
      View in: PubMed
    28. Pinotsis N, Lange S, Perriard JC, Svergun DI, Wilmanns M. Molecular basis of the C-terminal tail-to-tail assembly of the sarcomeric filament protein myomesin. EMBO J. 2008 Jan 09; 27(1):253-64. PMID: 18059477.
      View in: PubMed
    29. Schoenauer R, Lange S, Hirschy A, Ehler E, Perriard JC, Agarkova I. Myomesin 3, a novel structural component of the M-band in striated muscle. J Mol Biol. 2008 Feb 15; 376(2):338-51. PMID: 18177667.
      View in: PubMed
    30. Sheikh F, Bang ML, Lange S, Chen J. "Z"eroing in on the role of Cypher in striated muscle function, signaling, and human disease. Trends Cardiovasc Med. 2007 Nov; 17(8):258-62. PMID: 18021935; PMCID: PMC2134983.
    31. Müller S, Lange S, Gautel M, Wilmanns M. Rigid conformation of an immunoglobulin domain tandem repeat in the A-band of the elastic muscle protein titin. J Mol Biol. 2007 Aug 10; 371(2):469-80. PMID: 17574571.
      View in: PubMed
    32. Pinotsis N, Petoukhov M, Lange S, Svergun D, Zou P, Gautel M, Wilmanns M. Evidence for a dimeric assembly of two titin/telethonin complexes induced by the telethonin C-terminus. J Struct Biol. 2006 Aug; 155(2):239-50. PMID: 16713295.
      View in: PubMed
    33. Zou P, Pinotsis N, Lange S, Song YH, Popov A, Mavridis I, Mayans OM, Gautel M, Wilmanns M. Palindromic assembly of the giant muscle protein titin in the sarcomeric Z-disk. Nature. 2006 Jan 12; 439(7073):229-33. PMID: 16407954.
      View in: PubMed
    34. Lange S, Ehler E, Gautel M. From A to Z and back? Multicompartment proteins in the sarcomere. Trends Cell Biol. 2006 Jan; 16(1):11-8. PMID: 16337382.
      View in: PubMed
    35. Lange S, Xiang F, Yakovenko A, Vihola A, Hackman P, Rostkova E, Kristensen J, Brandmeier B, Franzen G, Hedberg B, Gunnarsson LG, Hughes SM, Marchand S, Sejersen T, Richard I, Edström L, Ehler E, Udd B, Gautel M. The kinase domain of titin controls muscle gene expression and protein turnover. Science. 2005 Jun 10; 308(5728):1599-603. PMID: 15802564.
      View in: PubMed
    36. Lange S, Himmel M, Auerbach D, Agarkova I, Hayess K, Fürst DO, Perriard JC, Ehler E. Dimerisation of myomesin: implications for the structure of the sarcomeric M-band. J Mol Biol. 2005 Jan 14; 345(2):289-98. PMID: 15571722.
      View in: PubMed
    37. Lange S, Agarkova I, Perriard JC, Ehler E. The sarcomeric M-band during development and in disease. J Muscle Res Cell Motil. 2005; 26(6-8):375-9. PMID: 16470337.
      View in: PubMed
    38. Agarkova I, Ehler E, Lange S, Schoenauer R, Perriard JC. M-band: a safeguard for sarcomere stability? J Muscle Res Cell Motil. 2003; 24(2-3):191-203. PMID: 14609030.
      View in: PubMed
    39. Lange S, Auerbach D, McLoughlin P, Perriard E, Schäfer BW, Perriard JC, Ehler E. Subcellular targeting of metabolic enzymes to titin in heart muscle may be mediated by DRAL/FHL-2. J Cell Sci. 2002 Dec 15; 115(Pt 24):4925-36. PMID: 12432079.
      View in: PubMed