Accelerating therapeutic development and clinical trial readiness for STXBP1 and SYNGAP1 disorders.
Marotta N, Boland MJ, Prosser BL.
Probl Pediatr Adolesc Health Care. 2024 Mar 11:101576. [Epub ahead of print]. doi: 10.1016/j.cppeds.2024.101576. PMID: 38472035. Review.
Integrated Stress Response Potentiates Ponatinib-Induced Cardiotoxicity.
Yan G, Han Z, Kwon Y, Jousma J, Nukala SB, Prosser BL, Du X, Pinho S, Ong SB, Lee WH, Ong SG.
Circ Res. 2024 Mar 1;134(5):482-501. PMID:38323474. doi: 10.1161/CIRCRESAHA.123.323683
Chronic activation of tubulin tyrosination in HCM mice and human iPSC-engineered heart tissues improves heart function.
Pietsch N, Chen CY, Kupsch S, Bacmeister L, Geertz B, Herera-Rivero M, Voß H, Krämer E, Braren I, Westermann D, Schlüter H, Mearini G, Schlossarek S, van der Velden J, Caporizzo MA, Lindner D, Prosser BL, Carrier L.
bioRxiv. 2024 Feb 7. PubMed PMID: 37292763. doi: 10.1101/2023.05.25.542365.
Truncated titin protein in dilated cardiomyopathy incorporates into the sarcomere and transmits force.
McAfee Q, Caporizzo MA, Uchida K, Bedi KC, Margulies KB, Arany Z, Prosser BL.
J Clin Invest. 2024 Jan 16;134(2):e170196. PMID: 37943622 doi: 10.1172/JCI170196.
Pinpointing the contributors to myocardial passive stiffness.
Prosser BL.
Nat. Cardiovas. Res., 2023 Oct 26. Epublished DOI: 10.1038/s44161-023-00350-7
Adult human cardiomyocyte mechanics in osteogenesis imperfecta
Lee, BW, Caporizzo MA, Chen CY, Bedi KC Jr, Peyster EG, Prosser BL, Margulies KB, Vite A.
Am J Physiol Heart Circ Physiol., 2023 Oct 1;325(4):H814-H821. Epub 2023 Aug 11. PMID: 37566108. doi: 10.1152/ajpheart.00391.2023.
An unbiased screen identified the Hsp70-BAG3 complex as a regulator of myosin binding protein C3
Thompson AD, Wagner MJ, Rodriguez J, Malhotra A, Vander Roest S, Lilienthal U, Shao H, Vignesh M, Weber K, Yob JM, Prosser BL, Helms AS, Gestwicki JE, Ginsburg D, Day SM.
J Am Coll Cardiol Basic Trans Science, 2023 Aug2;8(9):1198-1211. Epublished DOI: 10.1016/j.jacbts.2023.04.009.
Early life seizures and epileptic spasms in STXBP1 -related disorders.
Thalwitzer KM, Xian J, deCampo D, Parthasarathy S, Magielski J, Sullivan KR, Goss J, Rigby CS, Boland M, Prosser B, Ruggiero SM, Syrbe S, Helbig I.
medRxiv. 2023 Jun 28:2023.06.26.23291892. doi: 10.1101/2023.06.26.23291892. Preprint.PMID: 37425705.
Delineating clinical and developmental outcomes in STXBP1-related disorders.
Xian J, Thalwitzer KM, McKee J, Sullivan KR, Brimble E, Fitch E, Toib J, Kaufman MC, deCampo D, Cunningham K, Pierce SR, Goss J, Son Rigby C, Syrbe S, Boland M, Prosser B, Fitter N, Ruggiero SM, Helbig I.
medRxiv. 2023 May 11:2023.05.10.23289776. Preprint. PMID: 37215006. doi: 10.1101/2023.05.10.23289776.
Mapping PTBP2 binding in human brain identifies SYNGAP1 as a target for therapeutic splice switching.
Dawicki-McKenna JM, Felix AJ, Waxman EA, Cheng C, Amado DA, Ranum PT, Bogush A, Dungan LV, Maguire JA, Gagne AL, Heller EA, French DL, Davidson BL, Prosser BL.
Nat Commun. 2023 May 6;14(1):2628. PMID: 37149717. doi: 10.1038/s41467-023-38273-3.
Big tau aggregation disrupts microtubule tyrosination and causes myocardial diastolic dysfunction: from discovery to therapy
Luciani M, Montalbano M, Troncone L, Bacchin C, Uchida K, Daniele G, Wolf BJ, Butler HM, Kiel J, Berto S, Gensemer C, Moore K, Morningstar J, Diteepeng T, Albayram O, Abisambra JF, Norris RA, Di Salvo TG, Prosser BL, Kayed R, del Monte F.
Eur Heart J, 2023 May 1;44(17):1560–1570. PMID: 37122097. doi: 10.1093/eurheartj/ehad205.
Desmin intermediate filaments and tubulin detyrosination stabilize growing microtubules in the cardiomyocyte.
Salomon AK, Phyo SA, Okami N, Heffler J, Robison P, Bogush AI, Prosser BL.
Basic Res Cardiol. 2022 Nov 3;117(1):53. PMID: 36326891. doi: 10.1007/s00395-022-00962-3.
Integrated landscape of cardiac metabolism in end-stage human nonischemic dilated cardiomyopathy.
Flam E, Jang C, Murashige D, Yang Y, Morley MP, Jung S, Kantner DS, Pepper H, Bedi KC Jr, Brandimarto J, Prosser BL, Cappola T, Snyder NW, Rabinowitz JD, Margulies KB, Arany Z.
Nat Cardiovasc Res. 2022 Sep;1(9):817-829. Epub 2022 Aug 29. PMID: 36776621. doi: 10.1038/s44161-022-00117-6.
Extracellular stiffness induces contractile dysfunction in adult cardiomyocytes via cell-autonomous and microtubule-dependent mechanisms.
Vite A, Caporizzo MA, Corbin EA, Brandimarto J, McAfee Q, Livingston CE, Prosser BL, Margulies KB.
Basic Res Cardiol. 2022 Aug 25;117(1):41. PMID: 36006489. doi: 10.1007/s00395-022-00952-5.
The microtubule cytoskeleton in cardiac mechanics and heart failure.
Caporizzo MA, Prosser BL.
Nat Rev Cardiol. 2022 Jun;19(6):364-378. Epub 2022 Apr 19. PMID: 35440741. doi: 10.1038/s41569-022-00692-y. Review.
Assessing the landscape of STXBP1-related disorders in 534 individuals.
Xian J, Parthasarathy S, Ruggiero SM, Balagura G, Fitch E, Helbig K, Gan J, Ganesan S, Kaufman MC, Ellis CA, Lewis-Smith D, Galer P, Cunningham K, O’Brien M, Cosico M, Baker K, Darling A, Veiga de Goes F, El Achkar CM, Doering JH, Furia F, García-Cazorla Á, Gardella E, Geertjens L, Klein C, Kolesnik-Taylor A, Lammertse H, Lee J, Mackie A, Misra-Isrie M, Olson H, Sexton E, Sheidley B, Smith L, Sotero L, Stamberger H, Syrbe S, Thalwitzer KM, van Berkel A, van Haelst M, Yuskaitis C, Weckhuysen S, Prosser B, Son Rigby C, Demarest S, Pierce S, Zhang Y, Møller RS, Bruining H, Poduri A, Zara F, Verhage M, Striano P, Helbig I.
Brain. 2022 Jun 3;145(5):1668-1683. PMID: 35190816. doi: 10.1093/brain/awab327.
Base editing the synapse: Modeling a complex neurological disorder in non-human primates.
Prosser BL, Helbig I.
Mol Ther. 2022 Jun 1;30(6):2114-2116. Epub 2022 May 24. PMID: 35613622. doi: 10.1016/j.ymthe.2022.05.009.
Transcriptional, Post-Transcriptional, and Post-Translational Mechanisms Rewrite the Tubulin Code During Cardiac Hypertrophy and Failure.
Phyo SA, Uchida K, Chen CY, Caporizzo MA, Bedi K, Griffin J, Margulies K, Prosser BL.
Front Cell Dev Biol. 2022 Apr 1;10:837486. eCollection 2022. PMID: 35433678. doi: 10.3389/fcell.2022.837486.
Truncated titin proteins in dilated cardiomyopathy.
McAfee Q, Chen CY, Yang Y, Caporizzo MA, Morley M, Babu A, Jeong S, Brandimarto J, Bedi KC Jr, Flam E, Cesare J, Cappola TP, Margulies K, Prosser B, Arany Z.
Sci Transl Med. 2021 Nov 3;13(618):eabd7287. Epub 2021 Nov 3.PMID: 34731015. doi: 10.1126/scitranslmed.abd7287.
Cardiomyocyte Microtubules: Control of Mechanics, Transport, and Remodeling.
Uchida K, Scarborough EA, Prosser BL.
Annu Rev Physiol. 2022 Feb 10;84:257-283. doi: 10.1146/annurev-physiol-062421-040656. Epub 2021 Oct 6. PMID: 34614374. Review.
Need for Speed: The Importance of Physiological Strain Rates in Determining Myocardial Stiffness.
Caporizzo MA, Prosser BL.
Front Physiol. 2021 Jul 30;12:696694. eCollection 2021. PMID: 34393820. doi: 10.3389/fphys.2021.696694.
X-ROS Signaling Depends on Length-Dependent Calcium Buffering by Troponin.
Limbu S, Prosser BL, Lederer WJ, Ward CW, Jafri MS.
Cells. 2021 May 13;10(5):1189. PMID: 34068012. doi: 10.3390/cells10051189.
Cochlear supporting cells require GAS2 for cytoskeletal architecture and hearing.
Chen T, Rohacek AM, Caporizzo M, Nankali A, Smits JJ, Oostrik J, Lanting CP, Kücük E, Gilissen C, van de Kamp JM, Pennings RJE, Rakowiecki SM, Kaestner KH, Ohlemiller KK, Oghalai JS, Kremer H, Prosser BL, Epstein DJ.
Dev Cell. 2021 May 17;56(10):1526-1540.e7. Epub 2021 May 7. PMID: 33964205. doi: 10.1016/j.devcel.2021.04.017.
Microtubules orchestrate local translation to enable cardiac growth.
Scarborough EA, Uchida K, Vogel M, Erlitzki N, Iyer M, Phyo SA, Bogush A, Kehat I, Prosser BL.
Nat Commun. 2021 Mar 11;12(1):1547. PMID: 33707436. doi: 10.1038/s41467-021-21685-4.
Pathogenic LMNA variants disrupt cardiac lamina-chromatin interactions and de-repress alternative fate genes.
Shah PP, Lv W, Rhoades JH, Poleshko A, Abbey D, Caporizzo MA, Linares-Saldana R, Heffler JG, Sayed N, Thomas D, Wang Q, Stanton LJ, Bedi K, Morley MP, Cappola TP, Owens AT, Margulies KB, Frank DB, Wu JC, Rader DJ, Yang W, Prosser BL, Musunuru K, Jain R.
Cell Stem Cell. 2021 May 6;28(5):938-954.e9. Epub 2021 Feb 1. PMID: 33529599. doi: 10.1016/j.stem.2020.12.016.
Tubulin Detyrosination: An Emerging Therapeutic Target in Hypertrophic Cardiomyopathy.
Margulies KB, Prosser BL.
Circ Heart Fail. 2021 Jan;14(1):e008006. Epub 2021 Jan 12. PMID: 33430601. doi: 10.1161/CIRCHEARTFAILURE.120.008006.
Depletion of Vasohibin 1 Speeds Contraction and Relaxation in Failing Human Cardiomyocytes.
Chen CY, Salomon AK, Caporizzo MA, Curry S, Kelly NA, Bedi K, Bogush AI, Krämer E, Schlossarek S, Janiak P, Moutin MJ, Carrier L, Margulies KB, Prosser BL.
Circ Res. 2020 Jul 3;127(2):e14-e27. Epub 2020 Apr 10. PMID: 32272864. doi: 10.1161/CIRCRESAHA.119.315947.
Microtubules Increase Diastolic Stiffness in Failing Human Cardiomyocytes and Myocardium.
Caporizzo MA, Chen CY, Bedi K, Margulies KB, Prosser BL.
Circulation. 2020 Mar 17;141(11):902-915. Epub 2020 Jan 16. PMID: 31941365. doi: 10.1161/CIRCULATIONAHA.119.043930.
A Balance Between Intermediate Filaments and Microtubules Maintains Nuclear Architecture in the Cardiomyocyte.
Heffler J, Shah PP, Robison P, Phyo S, Veliz K, Uchida K, Bogush A, Rhoades J, Jain R, Prosser BL.
Circ Res. 2020 Jan 31;126(3):e10-e26. Epub 2019 Dec 11. PMID: 31822208. doi: 10.1161/CIRCRESAHA.119.315582.
Cardiac microtubules in health and heart disease.
Caporizzo MA, Chen CY, Prosser BL.
Exp Biol Med. 2019 Nov;244(15):1255-1272. Epub 2019 Aug 9. PMID: 31398994. doi: 10.1177/1535370219868960. Review.
Mechanosensing by the Lamina Protects against Nuclear Rupture, DNA Damage, and Cell-Cycle Arrest.
Cho S, Vashisth M, Abbas A, Majkut S, Vogel K, Xia Y, Ivanovska IL, Irianto J, Tewari M, Zhu K, Tichy ED, Mourkioti F, Tang HY, Greenberg RA, Prosser BL, Discher DE.
Dev Cell. 2019 Jun 17;49(6):920-935.e5. Epub 2019 May 16. PMID: 31105008. doi: 10.1016/j.devcel.2019.04.020.
Microtubules Provide a Viscoelastic Resistance to Myocyte Motion.
Caporizzo MA, Chen CY, Salomon AK, Margulies KB, Prosser BL.
Biophys J. 2018 Nov 6;115(9):1796-1807. Epub 2018 Sep 28. PMID: 30322798.doi: 10.1016/j.bpj.2018.09.019.
Suppression of detyrosinated microtubules improves cardiomyocyte function in human heart failure.
Chen CY, Caporizzo MA, Bedi K, Vite A, Bogush AI, Robison P, Heffler JG, Salomon AK, Kelly NA, Babu A, Morley MP, Margulies KB, Prosser BL.
Nat Med. 2018 Aug;24(8):1225-1233. Epub 2018 Jun 11. PMID: 29892068. doi: 10.1038/s41591-018-0046-2.
MIRO-1 Determines Mitochondrial Shape Transition upon GPCR Activation and Ca2+ Stress.
Nemani N, Carvalho E, Tomar D, Dong Z, Ketschek A, Breves SL, Jaña F, Worth AM, Heffler J, Palaniappan P, Tripathi A, Subbiah R, Riitano MF, Seelam A, Manfred T, Itoh K, Meng S, Sesaki H, Craigen WJ, Rajan S, Shanmughapriya S, Caplan J, Prosser BL, Gill DL, Stathopulos PB, Gallo G, Chan DC, Mishra P, Madesh M.
Cell Rep. 2018 Apr 24;23(4):1005-1019. PMID: 29694881. doi: 10.1016/j.celrep.2018.03.098.
Microtubule mechanics in the working myocyte.
Robison P, Prosser BL.
J Physiol. 2017 Jun 15;595(12):3931-3937. Epub 2017 Mar 9.PMID: 28116814. doi: 10.1113/JP273046. Review.
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. eCollection 2017 Jun. PMID: 28630914. doi: 10.1126/sciadv.1603081.
Mechanical signaling coordinates the embryonic heartbeat.
Chiou KK, Rocks JW, Chen CY, Cho S, Merkus KE, Rajaratnam A, Robison P, Tewari M, Vogel K, Majkut SF, Prosser BL, Discher DE, Liu AJ.
Proc Natl Acad Sci U S A. 2016 Aug 9;113(32):8939-44. Epub 2016 Jul 25. PMID: 27457951. doi: 10.1073/pnas.1520428113.
Detyrosinated microtubules buckle and bear load in contracting cardiomyocytes.
Robison P, Caporizzo MA, Ahmadzadeh H, Bogush AI, Chen CY, Margulies KB, Shenoy VB, Prosser BL.
Science. 2016 Apr 22;352(6284):aaf0659. PMID: 27102488. doi: 10.1126/science.aaf0659.
Modeling Local X-ROS and Calcium Signaling in the Heart.
Limbu S, Hoang-Trong TM, Prosser BL, Lederer WJ, Jafri MS.
Biophys J. 2015 Nov 17;109(10):2037-50. PMID: 26588563. doi: 10.1016/j.bpj.2015.09.031.
Detyrosinated microtubules modulate mechanotransduction in heart and skeletal muscle.
Kerr JP, Robison P, Shi G, Bogush AI, Kempema AM, Hexum JK, Becerra N, Harki DA, Martin SS, Raiteri R, Prosser BL, Ward CW.
Nat Commun. 2015 Oct 8;6:8526. PMID: 26446751. doi: 10.1038/ncomms9526.
Contractile Function During Angiotensin-II Activation: Increased Nox2 Activity Modulates Cardiac Calcium Handling via Phospholamban Phosphorylation.
Zhang M, Prosser BL, Bamboye MA, Gondim ANS, Santos CX, Martin D, Ghigo A, Perino A, Brewer AC, Ward CW, Hirsch E, Lederer WJ, Shah AM.
J Am Coll Cardiol. 2015 Jul 21;66(3):261-272. PMID: 26184620. doi: 10.1016/j.jacc.2015.05.020.
Myosin-binding protein C corrects an intrinsic inhomogeneity in cardiac excitation-contraction coupling.
Previs MJ, Prosser BL, Mun JY, Previs SB, Gulick J, Lee K, Robbins J, Craig R, Lederer WJ, Warshaw DM.
Sci Adv. 2015;1(1):e1400205. PMID: 25839057. doi: 10.1126/sciadv.1400205.
Mechano-chemo transduction tunes the heartstrings.
Prosser BL, Ward CW.
Sci Signal. 2014 Mar 18;7(317):pe7. PMID: 24643798. doi: 10.1126/scisignal.2005214.
Mechanical stretch-induced activation of ROS/RNS signaling in striated muscle.
Ward CW, Prosser BL, Lederer WJ.
Antioxid Redox Signal. 2014 Feb 20;20(6):929-36. Epub 2014 Jan 3. PMID: 23971496. doi: 10.1089/ars.2013.5517. Review.
Meeting highlights from the 2013 European Society of Cardiology Heart Failure Association Winter Meeting on Translational Heart Failure Research
Hohl M, Ardehali H, Azuaje FJ, Breckenridge RA, Doehner W, Eaton P, Ehret GB, Fujita T, Gaetani R, Giacca M, Hasenfuß G, Heymans S, Leite-Moreira AF, Linke WA, Linz D, Lyon A, Mamas MA, Orešič M, Papp Z, Pedrazzini T, Piepoli M, Prosser BL, Rizzuto R, Tarone G, Tian R, van Craenenbroeck E, van Rooij E, Wai T, Weiss G, Maack C.
Eur J Heart Fail. 2014 Jan;16(1):6-14. Epub 2013 Dec 14. PMID: 24453095. doi: 10.1002/ejhf.10. Review.
X-ROS signalling is enhanced and graded by cyclic cardiomyocyte stretch.
Prosser BL, Ward CW, Lederer WJ.
Cardiovasc Res. 2013 May 1;98(2):307-14. Epub 2013 Mar 21. PMID: 23524301. doi: 10.1093/cvr/cvt066.
X-ROS signaling in the heart and skeletal muscle: stretch-dependent local ROS regulates [Ca²⁺]i.
Prosser BL, Khairallah RJ, Ziman AP, Ward CW, Lederer WJ.
J Mol Cell Cardiol. 2013 May;58:172-81. Epub 2012 Dec 6. PMID: 23220288. doi: 10.1016/j.yjmcc.2012.11.011. Review.
Microtubules underlie dysfunction in Duchenne muscular dystrophy.
Khairallah RJ, Shi G, Sbrana F, Prosser BL, Borroto C, Mazaitis MJ, Hoffman EP, Mahurkar A, Sachs F, Sun Y, Chen YW, Raiteri R, Lederer WJ, Dorsey SG, Ward CW.
Sci Signal. 2012 Aug 7;5(236):ra56. PMID: 22871609. doi: 10.1126/scisignal.2002829.
X-ROS signaling: rapid mechano-chemo transduction in heart.
Prosser BL, Ward CW, Lederer WJ.
Science. 2011 Sep 9;333(6048):1440-5. PMID: 21903813. doi: 10.1126/science.1202768.
S100A1 and calmodulin regulation of ryanodine receptor in striated muscle.
Prosser BL, Hernández-Ochoa EO, Schneider MF.
Cell Calcium. 2011 Oct;50(4):323-31. Epub 2011 Jul 23. PMID: 21784520. doi: 10.1016/j.ceca.2011.06.001. Review.
Modulation of sarcoplasmic reticulum Ca2+ release in skeletal muscle expressing ryanodine receptor impaired in regulation by calmodulin and S100A1.
Yamaguchi N, Prosser BL, Ghassemi F, Xu L, Pasek DA, Eu JP, Hernández-Ochoa EO, Cannon BR, Wilder PT, Lovering RM, Weber D, Melzer W, Schneider MF, Meissner G.
Am J Physiol Cell Physiol. 2011 May;300(5):C998-C1012. Epub 2011 Feb 2. PMID: 21289290. doi: 10.1152/ajpcell.00370.2010.
Physiology, structure, and susceptibility to injury of skeletal muscle in mice lacking keratin 19-based and desmin-based intermediate filaments.
Lovering RM, O’Neill A, Muriel JM, Prosser BL, Strong J, Bloch RJ.
Am J Physiol Cell Physiol. 2011 Apr;300(4):C803-13. Epub 2011 Jan 5. PMID: 21209367. doi: 10.1152/ajpcell.00394.2010.
S100A1 promotes action potential-initiated calcium release flux and force production in skeletal muscle.
Prosser BL, Hernández-Ochoa EO, Lovering RM, Andronache Z, Zimmer DB, Melzer W, Schneider MF.
Am J Physiol Cell Physiol. 2010 Nov;299(5):C891-902. Epub 2010 Aug 4. PMID: 20686070. doi: 10.1152/ajpcell.00180.2010.
Subcellular Ca2+ signaling in the heart: the role of ryanodine receptor sensitivity.
Prosser BL, Ward CW, Lederer WJ.
J Gen Physiol. 2010 Aug;136(2):135-42. PMID: 20660656. doi: 10.1085/jgp.201010406.
Augmentation of Cav1 channel current and action potential duration after uptake of S100A1 in sympathetic ganglion neurons.
Hernández-Ochoa EO, Prosser BL, Wright NT, Contreras M, Weber DJ, Schneider MF.
Am J Physiol Cell Physiol. 2009 Oct;297(4):C955-70. Epub 2009 Aug 5. PMID: 19657060. doi: 10.1152/ajpcell.00140.2009.
The Qgamma component of intra-membrane charge movement is present in mammalian muscle fibres, but suppressed in the absence of S100A1.
Prosser BL, Hernández-Ochoa EO, Zimmer DB, Schneider MF.
>J Physiol. 2009 Sep 15;587(Pt 18):4523-41. Epub 2009 Aug 3. PMID: 19651767. doi: 10.1113/jphysiol.2009.177238.
Simultaneous recording of intramembrane charge movement components and calcium release in wild-type and S100A1-/- muscle fibres.
Prosser BL, Hernández-Ochoa EO, Zimmer DB, Schneider MF.
J Physiol. 2009 Sep 15;587(Pt 18):4543-59. Epub 2009 Aug 3. PMID: 19651766. doi: 10.1113/jphysiol.2009.177246.
S100A1 and calmodulin compete for the same binding site on ryanodine receptor.
Wright NT, Prosser BL, Varney KM, Zimmer DB, Schneider MF, Weber DJ.
J Biol Chem. 2008 Sep 26;283(39):26676-83. Epub 2008 Jul 23. PMID: 18650434. doi: 10.1074/jbc.M804432200.
S100A1 binds to the calmodulin-binding site of ryanodine receptor and modulates skeletal muscle excitation-contraction coupling.
Prosser BL, Wright NT, Hernãndez-Ochoa EO, Varney KM, Liu Y, Olojo RO, Zimmer DB, Weber DJ, Schneider MF.
J Biol Chem. 2008 Feb 22;283(8):5046-57. Epub 2007 Dec 17. PMID: 18089560. doi: 10.1074/jbc.M709231200.