FRANCESCO B RAMIREZ

Summary

Publications

  1. doi request reprint Fibrillin-rich microfibrils - structural and instructive determinants of mammalian development and physiology
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, New Jersey, USA
    Connect Tissue Res 49:1-6. 2008
  2. ncbi request reprint Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, New York 08901, USA
    J Cell Physiol 213:326-30. 2007
  3. ncbi request reprint Marfan syndrome: from molecular pathogenesis to clinical treatment
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, 89 French Street, New Brunswick, NJ 08901, USA corrected
    Curr Opin Genet Dev 17:252-8. 2007
  4. ncbi request reprint Transcriptional regulation of the human alpha2(I) collagen gene (COL1A2), an informative model system to study fibrotic diseases
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, NJ 08901, USA
    Matrix Biol 25:365-72. 2006
  5. pmc Fibrillins 1 and 2 perform partially overlapping functions during aortic development
    Luca Carta
    Laboratory of Genetics and Organogenesis, Hospital for Special Surgery, The Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 281:8016-23. 2006
  6. pmc Esophageal muscle physiology and morphogenesis require assembly of a collagen XIX-rich basement membrane zone
    Hideaki Sumiyoshi
    Research Division of the Hospital for Special Surgery at the Weill College of Medicine of Cornell University, New York, NY 10019, USA
    J Cell Biol 166:591-600. 2004
  7. doi request reprint Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome
    Wanfen Xiong
    Department of Surgery, University of Nebraska Medical Center, Omaha, NE 68198, USA
    J Vasc Surg 47:166-72; discussion 172. 2008
  8. ncbi request reprint Magnetic resonance microscopy quantifies the disease progression in Marfan syndrome mice
    Vitalii V Itskovich
    Imaging Science Laboratories, Mount Sinai School of Medicine, New York, New York 10029 6574, USA
    J Magn Reson Imaging 17:435-9. 2003
  9. ncbi request reprint Cell signaling events: a view from the matrix
    Francesco Ramirez
    Laboratory of Genetics and Organogenesis Hospital for Special Surgery, Weill Medical College of Cornell University, 535 East 70th Street, New York, NY 10021, USA
    Matrix Biol 22:101-7. 2003
  10. pmc Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states
    Ronald D Cohn
    McKusick Nathans Institute of Genetic Medicine, Baltimore, Maryland 21205, USA
    Nat Med 13:204-10. 2007

Research Grants

  1. Pathophysiology of Basement Membrane Zone Collagens
    Francesco Ramirez; Fiscal Year: 2007
  2. ARCHITECTURAL MICROFIBRILS IN BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2009
  3. Pathophysiology of Basement Membrane Zone Collagens
    FRANCESCO B RAMIREZ; Fiscal Year: 2010
  4. CHARACTERIZATION OF EXTRACELLULAR MICROFIBRILS
    Francesco Ramirez; Fiscal Year: 2004
  5. NOVEL MICROFIBRILLAR COMPONENT
    Francesco Ramirez; Fiscal Year: 1999
  6. COLLAGEN XI ISOFORMS IN CARTILAGE AND BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2001
  7. MOLECULAR MECHANISMS OF ETHANOL INDUCED LIVER FIBROGENES
    Francesco Ramirez; Fiscal Year: 2002
  8. ARCHITECTURAL MICROFIBRILS IN BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2009

Collaborators

  • Barry Starcher
  • Harry C Dietz
  • Luca Carta
  • Ronald D Cohn
  • Jennifer P Habashi
  • Enid R Neptune
  • Wanfen Xiong
  • Kevin B Jones
  • Bart L Loeys
  • Tammy M Holm
  • Daniel P Judge
  • Erin C Klein
  • Loretha Myers
  • Lynn Y Sakai
  • Hélène Chanut-Delalande
  • Hideaki Sumiyoshi
  • Shizuko Tanaka
  • Vitalii V Itskovich
  • Manuel Koch
  • B Timothy Baxter
  • Rebecca A Knispel
  • Keith H Bridwell
  • Arshia A Soleimani
  • Paul D Sponseller
  • Christel Van Erp
  • Sabine Kost-Byerly
  • Christopher W Ward
  • Lynn Sakai
  • Matthew Gamradt
  • Linda Sandell
  • Gurkan Erkula
  • Matthew T Lisi
  • Colette M ap Rhys
  • Lygia Pereira
  • Marina Sukoyan
  • Djahida Bedja
  • Timothy K Cooper
  • Brett Lenart
  • Carla Calvi
  • Emilio Arteaga-Solis
  • Guosheng Liu
  • Daniel B Rifkin
  • Marc K Halushka
  • Douglas R Keene
  • Megan Podowski
  • Christian A Merkel
  • Noriko Hazeki
  • Kathleen Gabrielson
  • David L Huso
  • Sui Y Lee-Arteaga
  • Alexander Kerkis
  • Bart Loeys
  • Sui Y Lee
  • Agnes Fichard
  • Marilyne Malbouyres
  • Florence Ruggiero
  • Christelle Bonod-Bidaud
  • Stephen Doty
  • Hidekatsu Yoshioka
  • Satish Rattan
  • Sylvain Cogne
  • Niv Mor
  • Scott Henderson
  • Donald R Gerecke
  • Dan E Arking
  • Robert E Burgeson
  • Marion K Gordon
  • Tracie E Bunton
  • Rita A Hahn
  • Mark Lieb
  • Friedrich Laub
  • Juan Gilberto S Aguinaldo
  • Peihong Zhou
  • Zahi A Fayad
  • Pamela A Frischmeyer
  • Daniel D Samber
  • Barbara Gayraud

Detail Information

Publications18

  1. doi request reprint Fibrillin-rich microfibrils - structural and instructive determinants of mammalian development and physiology
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, New Jersey, USA
    Connect Tissue Res 49:1-6. 2008
    ..This review summarizes the evidence supporting our current understanding of the structural and instructive roles that fibrillin-rich microfibrils play during embryonic development and in human diseases...
  2. ncbi request reprint Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, New York 08901, USA
    J Cell Physiol 213:326-30. 2007
    ..This review summarizes our current understanding of the role of fibrillin-rich microfibrils in development and disease, as well as exciting new applications in the clinical management of MFS and related connective tissue disorders...
  3. ncbi request reprint Marfan syndrome: from molecular pathogenesis to clinical treatment
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, 89 French Street, New Brunswick, NJ 08901, USA corrected
    Curr Opin Genet Dev 17:252-8. 2007
    ..More generally, these studies have established that Marfan syndrome is part of a group of developmental disorders with broad and complex effects on morphogenesis, homeostasis and organ function...
  4. ncbi request reprint Transcriptional regulation of the human alpha2(I) collagen gene (COL1A2), an informative model system to study fibrotic diseases
    Francesco Ramirez
    Child Health Institute of New Jersey, Robert W Johnson Medical School, New Brunswick, NJ 08901, USA
    Matrix Biol 25:365-72. 2006
    ..These transcriptional studies have also an additional value in translational research, in that they are providing the conceptual means to develop new animal models of and therapeutic strategies for fibrotic diseases...
  5. pmc Fibrillins 1 and 2 perform partially overlapping functions during aortic development
    Luca Carta
    Laboratory of Genetics and Organogenesis, Hospital for Special Surgery, The Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 281:8016-23. 2006
    ....
  6. pmc Esophageal muscle physiology and morphogenesis require assembly of a collagen XIX-rich basement membrane zone
    Hideaki Sumiyoshi
    Research Division of the Hospital for Special Surgery at the Weill College of Medicine of Cornell University, New York, NY 10019, USA
    J Cell Biol 166:591-600. 2004
    ..Therefore, these findings identify collagen XIX as the first structural determinant of sphincteric muscle function, and as the first extrinsic factor of skeletal myogenesis in the murine esophagus...
  7. doi request reprint Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome
    Wanfen Xiong
    Department of Surgery, University of Nebraska Medical Center, Omaha, NE 68198, USA
    J Vasc Surg 47:166-72; discussion 172. 2008
    ..Doxycycline is a nonspecific MMP inhibitor. The objective of the study was to determine whether docycycline can attenuate matrix degradation and prolong the survival of mice with MFS...
  8. ncbi request reprint Magnetic resonance microscopy quantifies the disease progression in Marfan syndrome mice
    Vitalii V Itskovich
    Imaging Science Laboratories, Mount Sinai School of Medicine, New York, New York 10029 6574, USA
    J Magn Reson Imaging 17:435-9. 2003
    ..To use noninvasive magnetic resonance microscopy (MRM) to examine aneurysmal disease in the mouse model of Marfan syndrome (MFS)...
  9. ncbi request reprint Cell signaling events: a view from the matrix
    Francesco Ramirez
    Laboratory of Genetics and Organogenesis Hospital for Special Surgery, Weill Medical College of Cornell University, 535 East 70th Street, New York, NY 10021, USA
    Matrix Biol 22:101-7. 2003
    ..These observations open the way to a novel understanding in these poorly understood and critically important areas of cell and developmental biology...
  10. pmc Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states
    Ronald D Cohn
    McKusick Nathans Institute of Genetic Medicine, Baltimore, Maryland 21205, USA
    Nat Med 13:204-10. 2007
    ..Moreover, we show TGF-beta-induced failure of muscle regeneration and a similar therapeutic response in a dystrophin-deficient mouse model of Duchenne muscular dystrophy...
  11. ncbi request reprint Symposium on the musculoskeletal aspects of Marfan syndrome: meeting report and state of the science
    Kevin B Jones
    Department of Orthopaedics and Rehabilitation, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, Iowa 52242, USA
    J Orthop Res 25:413-22. 2007
    ..Areas covered include clinical detection, diagnosis, growth, spine deformity, molecular mechanisms, dural ectasia, protrusio acetabuli, and pain in Marfan syndrome...
  12. ncbi request reprint Collagen XXIV, a vertebrate fibrillar collagen with structural features of invertebrate collagens: selective expression in developing cornea and bone
    Manuel Koch
    Institute for Biochemistry II, University of Cologne, Joseph Stelzmann Strasse 52, Cologne 50931, Germany
    J Biol Chem 278:43236-44. 2003
    ..Based on these data, we propose that collagen XXIV is an ancient molecule that may contribute to the regulation of type I collagen fibrillogenesis at specific anatomical locations during fetal development...
  13. pmc Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome
    Jennifer P Habashi
    Howard Hughes Medical Institute and Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Science 312:117-21. 2006
    ....
  14. pmc Development of a functional skin matrix requires deposition of collagen V heterotrimers
    Hélène Chanut-Delalande
    Institut de Biologie et Chimie des Proteines, UMR CNRS 5086, IFR128 Biosciences Lyon Gerland, France
    Mol Cell Biol 24:6049-57. 2004
    ....
  15. ncbi request reprint Recent progress towards a molecular understanding of Marfan syndrome
    Harry C Dietz
    Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Am J Med Genet C Semin Med Genet 139:4-9. 2005
    ..Importantly, new biological targets have emerged that may yield exciting new opportunities for the development of productive treatment strategies in MFS...
  16. ncbi request reprint Therapy insight: aortic aneurysm and dissection in Marfan's syndrome
    Francesco Ramirez
    Laboratory of Genetics and Organogenesis, Hospital for Special Surgery at the Weill Medical College of Cornell University, 535 East 70th Street, New York, NY 10021, USA
    Nat Clin Pract Cardiovasc Med 1:31-6. 2004
    ..As a result, exciting new hypotheses have emerged regarding the pathogenesis of MFS, as well as opportunities to explore translational applications of this information that may be relevant to various manifestations of the disease...
  17. ncbi request reprint Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology
    Francesco Ramirez
    Laboratory of Genetics and Organogenesis, Hospital for Special Surgery, and Department of Physiology and Biophysics, Weill Medical College of Cornell University, New York, New York 10021, USA
    Physiol Genomics 19:151-4. 2004
    ..These observations pave the way to novel therapeutic approaches aimed at counteracting the deleterious consequences of perturbations of connective tissue homeostasis...
  18. ncbi request reprint Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome
    Enid R Neptune
    Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nat Genet 33:407-11. 2003
    ..These data indicate that matrix sequestration of cytokines is crucial to their regulated activation and signaling and that perturbation of this function can contribute to the pathogenesis of disease...

Research Grants25

  1. Pathophysiology of Basement Membrane Zone Collagens
    Francesco Ramirez; Fiscal Year: 2007
    ....
  2. ARCHITECTURAL MICROFIBRILS IN BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2009
    ....
  3. Pathophysiology of Basement Membrane Zone Collagens
    FRANCESCO B RAMIREZ; Fiscal Year: 2010
    ....
  4. CHARACTERIZATION OF EXTRACELLULAR MICROFIBRILS
    Francesco Ramirez; Fiscal Year: 2004
    ..Collectively, it is anticipated that this multidisciplinary IRPG will yield an integrated and comprehensive view of bone modeling and remodeling. ..
  5. NOVEL MICROFIBRILLAR COMPONENT
    Francesco Ramirez; Fiscal Year: 1999
    ....
  6. COLLAGEN XI ISOFORMS IN CARTILAGE AND BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2001
    ..It is suggested (by the applicant) that this information will advance our understanding of the pathogenesis of disorders that impair skeletal function. ..
  7. MOLECULAR MECHANISMS OF ETHANOL INDUCED LIVER FIBROGENES
    Francesco Ramirez; Fiscal Year: 2002
    ..abstract_text> ..
  8. ARCHITECTURAL MICROFIBRILS IN BONE PHYSIOLOGY
    Francesco Ramirez; Fiscal Year: 2009
    ....