Intrinsic Gata4 expression sensitizes the aortic root to dilation in a Loeys-Dietz syndrome mouse model.

Autor: Bramel EE; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; Predoctoral Training in Human Genetics and Genomics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Camejo WAE; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; Predoctoral Training in Human Genetics and Genomics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Creamer TJ; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Restrepo L; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Saqib M; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Bagirzadeh R; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Zeng A; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Mitchell JT; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; Predoctoral Training in Human Genetics and Genomics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Stein-O'Brien GL; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., Pedroza AJ; Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, California, USA., Fischbein MP; Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, California, USA., Dietz HC; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA., MacFarlane EG; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Publikováno v: Research square [Res Sq] 2024 Jun 05. Date of Electronic Publication: 2024 Jun 05.

Postnatal Smad3 Inactivation in Murine Smooth Muscle Cells Elicits a Temporally and Regionally Distinct Transcriptional Response.

Autor: Bramel EE; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.; Predoctoral Training in Human Genetics and Molecular Biology, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Creamer TJ; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Saqib M; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Camejo Nunez WA; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.; Predoctoral Training in Human Genetics and Molecular Biology, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Bagirzadeh R; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Roker LA; School of Medicine Microscope Facility, Johns Hopkins University School of Medicine, Baltimore, MD, United States., Goff LA; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.; Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, United States.; Kavli Neuroscience Discovery Institute, Johns Hopkins University School of Medicine, Baltimore, MD, United States., MacFarlane EG; McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.; Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, United States.

Publikováno v: Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2022 Apr 08; Vol. 9, pp. 826495. Date of Electronic Publication: 2022 Apr 08 (Print Publication: 2022).

Calpain 9 as a therapeutic target in TGFβ-induced mesenchymal transition and fibrosis.

Autor: Kim DH; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.; Cellular and Molecular Medicine Program, School of Medicine, Baltimore, MD 21205, USA., Beckett JD; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Nagpal V; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Seman-Senderos MA; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.; Cellular and Molecular Medicine Program, School of Medicine, Baltimore, MD 21205, USA., Gould RA; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.; Meinig School of Biomedical Engineering, Cornell University, Ithaca, NY 14853, USA., Creamer TJ; Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., MacFarlane EG; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.; Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Chen Y; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Bedja D; Department of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Butcher JT; Meinig School of Biomedical Engineering, Cornell University, Ithaca, NY 14853, USA., Mitzner W; Department of Environmental Health Sciences, Johns Hopkins University Bloomberg School of Public Health, Baltimore, MD 21205, USA., Rouf R; Department of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Hata S; Department of Advanced Science for Biomolecules, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan., Warren DS; Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA., Dietz HC; McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. hdietz@jhmi.edu.; Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA.

Publikováno v: Science translational medicine [Sci Transl Med] 2019 Jul 17; Vol. 11 (501).

Oxytocin antagonism prevents pregnancy-associated aortic dissection in a mouse model of Marfan syndrome.

Autor: Habashi JP; Division of Pediatric Cardiology, Johns Hopkins University, Baltimore, MD 21287, USA., MacFarlane EG; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Bagirzadeh R; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Bowen C; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Huso N; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Chen Y; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Bedja D; Division of Comparative Medicine, Johns Hopkins University, Baltimore, MD 21287, USA., Creamer TJ; Department of Surgery, Johns Hopkins University, Baltimore, MD 21287, USA., Rykiel G; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA., Manning M; Department of Cancer Biology, University of Toledo College of Medicine, Toledo, OH 43614, USA., Huso D; Division of Comparative Medicine, Johns Hopkins University, Baltimore, MD 21287, USA., Dietz HC; Center for Medical Genetics, Johns Hopkins University, Baltimore, MD 21287, USA. hdietz@jhmi.edu.; Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA.

Publikováno v: Science translational medicine [Sci Transl Med] 2019 May 01; Vol. 11 (490).

Decreased mitochondrial respiration in aneurysmal aortas of Fibulin-4 mutant mice is linked to PGC1A regulation.

Autor: van der Pluijm I; Department of Vascular Surgery, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Burger J; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Clinical Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., van Heijningen PM; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., IJpma A; Clinical Bioinformatics Unit, Department of Pathology, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., van Vliet N; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Milanese C; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Schoonderwoerd K; Department of Clinical Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Sluiter W; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Ringuette LJ; Department of Anatomy and Cell Biology, McGill University, Rue University, Montréal, QC H3A 0C7, Canada., Dekkers DHW; Proteomics Center, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Que I; Department of Radiology, Leiden University Medical Center, Albinusdreef 2, ZA Leiden, The Netherlands., Kaijzel EL; Department of Radiology, Leiden University Medical Center, Albinusdreef 2, ZA Leiden, The Netherlands., Te Riet L; Department of Vascular Surgery, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Pharmacology, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., MacFarlane EG; Department of Surgery, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, USA., Das D; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., van der Linden R; Stem cell Institute, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Vermeij M; Department of Pathology, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Demmers JA; Proteomics Center, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Mastroberardino PG; Department of Molecular Genetics, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands., Davis EC; Department of Anatomy and Cell Biology, McGill University, Rue University, Montréal, QC H3A 0C7, Canada., Yanagisawa H; Life Science Center, Tsukuba Advanced Research Alliance, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, Japan., Dietz HC; Department of Surgery, McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, USA.; Institute of Genetic Medicine, Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, USA.; Division of Pediatric Cardiology, Department of Pediatrics, and Department of Medicine, Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, USA., Kanaar R; Department of Radiation Oncology, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Molecular Genetics, Oncode Institute, Erasmus MC, Rotterdan, The Netherlands., Essers J; Department of Vascular Surgery, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Radiation Oncology, Erasmus MC, Wytemaweg 80, CN Rotterdam, The Netherlands.; Department of Molecular Genetics, Oncode Institute, Erasmus MC, Rotterdan, The Netherlands.

Publikováno v: Cardiovascular research [Cardiovasc Res] 2018 Nov 01; Vol. 114 (13), pp. 1776-1793.