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Incomplete-penetrant hypertrophic cardiomyopathy MYH7 G256E mutation causes hypercontractility and elevated mitochondrial respiration.
Lee S, Vander Roest AS, Blair CA, Kao K, Bremner SB, Childers MC, Pathak D, Heinrich P, Lee D, Chirikian O, Mohran SE, Roberts B, Smith JE, Jahng JW, Paik DT, Wu JC, Gunawardane RN, Ruppel KM, Mack DL, Pruitt BL, Regnier M, Wu SM, Spudich JA, Bernstein D. Lee S, et al. Among authors: bernstein d. Proc Natl Acad Sci U S A. 2024 May 7;121(19):e2318413121. doi: 10.1073/pnas.2318413121. Epub 2024 Apr 29. Proc Natl Acad Sci U S A. 2024. PMID: 38683993
Hypertrophic cardiomyopathy β-cardiac myosin mutation (P710R) leads to hypercontractility by disrupting super relaxed state.
Vander Roest AS, Liu C, Morck MM, Kooiker KB, Jung G, Song D, Dawood A, Jhingran A, Pardon G, Ranjbarvaziri S, Fajardo G, Zhao M, Campbell KS, Pruitt BL, Spudich JA, Ruppel KM, Bernstein D. Vander Roest AS, et al. Among authors: bernstein d. Proc Natl Acad Sci U S A. 2021 Jun 15;118(24):e2025030118. doi: 10.1073/pnas.2025030118. Proc Natl Acad Sci U S A. 2021. PMID: 34117120 Free PMC article.
Altered Cardiac Energetics and Mitochondrial Dysfunction in Hypertrophic Cardiomyopathy.
Ranjbarvaziri S, Kooiker KB, Ellenberger M, Fajardo G, Zhao M, Vander Roest AS, Woldeyes RA, Koyano TT, Fong R, Ma N, Tian L, Traber GM, Chan F, Perrino J, Reddy S, Chiu W, Wu JC, Woo JY, Ruppel KM, Spudich JA, Snyder MP, Contrepois K, Bernstein D. Ranjbarvaziri S, et al. Among authors: bernstein d. Circulation. 2021 Nov 23;144(21):1714-1731. doi: 10.1161/CIRCULATIONAHA.121.053575. Epub 2021 Oct 21. Circulation. 2021. PMID: 34672721 Free PMC article.
Multi-scale models reveal hypertrophic cardiomyopathy MYH7 G256E mutation drives hypercontractility and elevated mitochondrial respiration.
Lee S, Roest ASV, Blair CA, Kao K, Bremner SB, Childers MC, Pathak D, Heinrich P, Lee D, Chirikian O, Mohran S, Roberts B, Smith JE, Jahng JW, Paik DT, Wu JC, Gunawardane RN, Spudich JA, Ruppel K, Mack D, Pruitt BL, Regnier M, Wu SM, Bernstein D. Lee S, et al. Among authors: bernstein d. bioRxiv [Preprint]. 2023 Jun 9:2023.06.08.544276. doi: 10.1101/2023.06.08.544276. bioRxiv. 2023. PMID: 37333118 Free PMC article. Updated. Preprint.
Improved Cardiac Performance and Decreased Arrhythmia in Hypertrophic Cardiomyopathy With Non-β-Blocking R-Enantiomer Carvedilol.
Seo K, Yamamoto Y, Kirillova A, Kawana M, Yadav S, Huang Y, Wang Q, Lane KV, Pruitt BL, Perez MV, Bernstein D, Wu JC, Wheeler MT, Parikh VN, Ashley EA. Seo K, et al. Among authors: bernstein d. Circulation. 2023 Nov 21;148(21):1691-1704. doi: 10.1161/CIRCULATIONAHA.123.065017. Epub 2023 Oct 18. Circulation. 2023. PMID: 37850394
Time-dependent evolution of functional vs. remodeling signaling in induced pluripotent stem cell-derived cardiomyocytes and induced maturation with biomechanical stimulation.
Jung G, Fajardo G, Ribeiro AJ, Kooiker KB, Coronado M, Zhao M, Hu DQ, Reddy S, Kodo K, Sriram K, Insel PA, Wu JC, Pruitt BL, Bernstein D. Jung G, et al. Among authors: bernstein d. FASEB J. 2016 Apr;30(4):1464-79. doi: 10.1096/fj.15-280982. Epub 2015 Dec 16. FASEB J. 2016. PMID: 26675706 Free PMC article.
PKA signaling shows a similar increase: 15.7 5.2% (d 30), 49.8 0.5% (d 60), and 71.0 6.1% (d 90). cAMP generation increases 9-fold from d 30 to 60, with enhanced coupling to remodeling pathways (e.g., Akt and Ca(2+)/calmodulin-dependent protein kinase …
PKA signaling shows a similar increase: 15.7 5.2% (d 30), 49.8 0.5% (d 60), and 71.0 6.1% (d 90). cAMP generation incre …
iPSC-derived cardiomyocytes reveal abnormal TGF-β signalling in left ventricular non-compaction cardiomyopathy.
Kodo K, Ong SG, Jahanbani F, Termglinchan V, Hirono K, InanlooRahatloo K, Ebert AD, Shukla P, Abilez OJ, Churko JM, Karakikes I, Jung G, Ichida F, Wu SM, Snyder MP, Bernstein D, Wu JC. Kodo K, et al. Among authors: bernstein d. Nat Cell Biol. 2016 Oct;18(10):1031-42. doi: 10.1038/ncb3411. Epub 2016 Sep 19. Nat Cell Biol. 2016. PMID: 27642787 Free PMC article.
1,897 results