Francesco Ramirez

Summary

Affiliation: Hospital for Special Surgery
Country: USA

Publications

  1. 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
  2. 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
  3. 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
  4. pmc Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10021, USA
    J Cell Biol 190:1107-21. 2010
  5. ncbi request reprint Identification of a repressor in the first intron of the human alpha2(I) collagen gene (COL1A2)
    Taras T Antoniv
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 280:35417-23. 2005
  6. pmc Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics at the Mount Sinai School of Medicine, New York, NY 10029, USA
    Hum Mol Genet 19:4790-8. 2010
  7. pmc Extracellular microfibrils control osteoblast-supported osteoclastogenesis by restricting TGF{beta} stimulation of RANKL production
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, New York 10021, USA
    J Biol Chem 285:34126-33. 2010
  8. pmc Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment
    Jason R Cook
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Genesis 50:635-41. 2012
  9. pmc Discrete contributions of elastic fiber components to arterial development and mechanical compliance
    Luca Carta
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Arterioscler Thromb Vasc Biol 29:2083-9. 2009
  10. doi request reprint Collagen XXIV (Col24a1) gene expression is a specific marker of osteoblast differentiation and bone formation
    Noritaka Matsuo
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, New York, New York, USA
    Connect Tissue Res 49:68-75. 2008

Collaborators

Detail Information

Publications43

  1. 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...
  2. 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...
  3. 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...
  4. pmc Fibrillin-1 and -2 differentially modulate endogenous TGF-β and BMP bioavailability during bone formation
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10021, USA
    J Cell Biol 190:1107-21. 2010
    ..Together, these findings identify the extracellular microfibrils as critical regulators of bone formation through the modulation of endogenous TGF-β and BMP signaling...
  5. ncbi request reprint Identification of a repressor in the first intron of the human alpha2(I) collagen gene (COL1A2)
    Taras T Antoniv
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, Weill Medical College of Cornell University, New York, New York 10021, USA
    J Biol Chem 280:35417-23. 2005
    ..More generally, the study reiterated the existence of species-specific difference in the regulatory networks of the mammalian alpha2(I) collagen coding genes...
  6. pmc Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics at the Mount Sinai School of Medicine, New York, NY 10029, USA
    Hum Mol Genet 19:4790-8. 2010
    ..Taken together, our findings shed new light on the pathogenesis of osteopenia in MFS, in addition to arguing for a multifaceted treatment strategy in this congenital disorder of the connective tissue...
  7. pmc Extracellular microfibrils control osteoblast-supported osteoclastogenesis by restricting TGF{beta} stimulation of RANKL production
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, New York 10021, USA
    J Biol Chem 285:34126-33. 2010
    ....
  8. pmc Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment
    Jason R Cook
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Genesis 50:635-41. 2012
    ..Fbn1(Lox/+) mice are, therefore, an informative genetic resource to further dissect MFS pathogenesis and the role of extracellular fibrillin-1 assemblies in organ development and homeostasis...
  9. pmc Discrete contributions of elastic fiber components to arterial development and mechanical compliance
    Luca Carta
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Arterioscler Thromb Vasc Biol 29:2083-9. 2009
    ..A genetic approach was therefore used here to distinguish the differential contributions of elastin and fibrillin-1 to arterial development and compliance...
  10. doi request reprint Collagen XXIV (Col24a1) gene expression is a specific marker of osteoblast differentiation and bone formation
    Noritaka Matsuo
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, New York, New York, USA
    Connect Tissue Res 49:68-75. 2008
    ..These findings lend further support to the hypothesis that collagen XXIV may be implicated in the formation of a mineralization-competent bone matrix...
  11. pmc p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice
    Luca Carta
    Department of Pharmacology and Systems Therapeutics and the Cardiovascular Institute, Mount Sinai School of Medicine, New York, New York 10029, USA
    J Biol Chem 284:5630-6. 2009
    ..Collectively, these findings indicate that improper activation of p38 MAPK is a precursor of constitutive Smad2/3 signaling in the aortic wall of a mouse model of neonatal lethal Marfan syndrome...
  12. ncbi request reprint The first intron of the human alpha2(I) collagen gene (COL1A2) contains a novel interferon-gamma responsive element
    Shizuko Tanaka
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, and Department Physiology and Biophysics at Weill Medical College of Cornell University, New York, NY 10021, USA
    Matrix Biol 26:185-9. 2007
    ..Together our findings further extend the list of transcription factors involved in INF-gamma inhibition of COL1A2 gene expression...
  13. ncbi request reprint GATA-4 binds to an upstream element of the human alpha2(I) collagen gene (COL1A2) and inhibits transcription in fibroblasts
    Lu Wang
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, Department Physiology and Biophysics at Weill Medical College of Cornell University, New York, NY 10021, USA
    Matrix Biol 24:333-40. 2005
    ..Finally, a chromatin immunoprecipitation assay documented GATA-4 ability to bind to the HS2 element in vivo. These results are therefore the first to implicate GATA-4 in regulating constitutive COL1A2 gene expression in fibroblasts...
  14. pmc Drug-based therapies for vascular disease in Marfan syndrome: from mouse models to human patients
    Jason R Cook
    Mount Sinai School of Medicine, New York, NY, USA
    Mt Sinai J Med 77:366-73. 2010
    ..More generally, information gathered from the study of Marfan syndrome pathogenesis has the potential to improve the clinical management of common acquired disorders of connective-tissue degeneration...
  15. pmc Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-beta activity
    Branka Dabovic
    Department of Cell Biology, New York University School of Medicine, New York, New York, USA
    J Cell Physiol 219:14-22. 2009
    ..Reversal of the air-sac septation defect was not associated with normalization of the elastogenesis indicating two separate functions of LTBP-4 as a regulator of elastic fiber assembly and TGF-beta levels in lungs...
  16. pmc Establishment of fibrillin-deficient osteoprogenitor cell lines identifies molecular abnormalities associated with extracellular matrix perturbation of osteogenic differentiation
    Silvia Smaldone
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, One Gustave L Levy Place, New York, NY 10029, USA
    Cell Tissue Res 344:511-7. 2011
    ..Collectively, the data reiterated the notion that fibrillin-1 and fibrillin-2 exert opposite effects on osteoblast differentiation through the discrete modulation of a broad network of interacting signaling molecules...
  17. 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
    ....
  18. pmc Biogenesis and function of fibrillin assemblies
    Francesco Ramirez
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY, USA
    Cell Tissue Res 339:71-82. 2010
    ....
  19. pmc Extracellular microfibrils: contextual platforms for TGFbeta and BMP signaling
    Francesco Ramirez
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Curr Opin Cell Biol 21:616-22. 2009
    ....
  20. doi request reprint Extracellular regulation of transforming growth factor beta and bone morphogenetic protein signaling in bone
    Harikiran Nistala
    Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY, USA
    Ann N Y Acad Sci 1192:253-6. 2010
    ..The latter finding is likely to improve the design of more effective therapeutic interventions in osteoporosis and of bioengineering formulations for the repair of bone fractures...
  21. pmc Developmental regulation of yolk sac hematopoiesis by Kruppel-like factor 6
    Nobuyuki Matsumoto
    Division of Liver Diseases, Department of Medicine, Mount Sinai School of Medicine, 1425 Madison Ave, Rm 1170F, New York, NY 10029, USA
    Blood 107:1357-65. 2006
    ..Forced expression of KLF6 using a tet-inducible system enhanced the hematopoietic potential of wild-type EBs. Collectively, these findings implicate Klf6 in ES-cell differentiation and hematopoiesis...
  22. ncbi request reprint CREB-AP1 protein complexes regulate transcription of the collagen XXIV gene (Col24a1) in osteoblasts
    Noritaka Matsuo
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, and Department of Physiology and Biophysics, Weill Medical College, Cornell University, New York, NY 10021, USA
    J Biol Chem 281:5445-52. 2006
    ....
  23. pmc Transcription factor KLF7 is important for neuronal morphogenesis in selected regions of the nervous system
    Friedrich Laub
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, and Department of Physiology and Biophysics at Weill Medical College of Cornell University, 535 East 70th St, New York, New York 10021, USA
    Mol Cell Biol 25:5699-711. 2005
    ..KLF7 activity might therefore control neuronal morphogenesis in part by optimizing the levels of molecules that promote axon outgrowth...
  24. pmc Ha-Ras stabilization mediates pro-fibrotic signals in dermal fibroblasts
    Silvia Smaldone
    Mount Sinai School of Medicine, Department of Pharmacology and Systems Therapeutics, One Gustave L Levy Place, Box 1603, New York, NY 10029, USA
    Fibrogenesis Tissue Repair 4:8. 2011
    ..abstract:..
  25. ncbi request reprint Cooperativity between far upstream enhancer and proximal promoter elements of the human {alpha}2(I) collagen (COL1A2) gene instructs tissue specificity in transgenic mice
    Shizuko Tanaka
    Laboratory of Genetics and Organogenesis, Research Division of the Hospital for Special Surgery, Weill Medical College of Cornell University, 535 East 70th Street, New York, NY 10021, USA
    J Biol Chem 279:56024-31. 2004
    ..This study thus reiterates the complex and highly combinatorial nature of the regulatory network governing COL1A2 transcription in vivo...
  26. pmc Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis
    Jacopo Olivieri
    Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, USA
    Fibrogenesis Tissue Repair 3:24. 2010
    ....
  27. 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)...
  28. doi request reprint Diagnostic and therapeutic strategies for small abdominal aortic aneurysms
    Ahmed Klink
    Translational and Molecular Imaging Institute, Mount Sinai School of Medicine, 1428 Madison Avenue, New York, NY 10029, USA
    Nat Rev Cardiol 8:338-47. 2011
    ..We also describe the diagnostic potential of new imaging techniques and therapeutic potential of new treatments for the management of small AAA...
  29. 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...
  30. 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
    ....
  31. 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...
  32. 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...
  33. 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...
  34. 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...
  35. 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...
  36. 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...
  37. 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...
  38. 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
    ....
  39. ncbi request reprint A report on the 3rd Workshop on Heritable Disorders of Connective Tissue
    Lynn Y Sakai
    Department of Biochemistry and Molecular Biology, Shriners Hospital for Children, Oregon Health Sciences University, 3101 SW Sam Jackson Park Road, Portland, OR 97201, USA
    Matrix Biol 21:7-13. 2002
    ..In addition to the invited participants, more than eighty guests (scientists, NIH staff, and members of the Coalition for Heritable Disorders of Connective Tissue) attended...
  40. 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...
  41. 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...
  42. pmc Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders
    Gail D Pearson
    National Heart, Lung, and Blood Institute, National Institutes of Health, Division of Cardiovascular Diseases, Bethesda, MD 20892, USA
    Circulation 118:785-91. 2008
  43. pmc Early response of alpha2(I) collagen to acetaldehyde in human hepatic stellate cells is TGF-beta independent
    Gianluca Svegliati-Baroni
    Clinica di Gastroenterologia, Universita Politecnica delle Marche, Ancona, Italy
    Hepatology 42:343-52. 2005
    ..We suggest that early acetaldehyde-dependent events induce the late expression of TGF-beta1 and create an H(2)O(2)-dependent autocrine loop that may sustain and amplify the fibrogenic response of this alcohol metabolite...