Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1

DOI Web Site 被引用文献19件
  • Lygia Pereira
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Sui Ying Lee
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Barbara Gayraud
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Kostantinos Andrikopoulos
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Steven D. Shapiro
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Tracie Bunton
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Nancy Jensen Biery
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Harry C. Dietz
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Lynn Y. Sakai
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...
  • Francesco Ramirez
    Brookdale Center for Developmental and Molecular Biology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Istituto de Biociencias, Departamento de Biologia, University of São Paulo, SP 05508 Brazil; Departments of Pediatrics, Medicine, and Cell Biology and Physiology, Washington University School of Medicine at Barnes–Jewish Hospital, St. Louis, MO 63110; Departments of Comparative Medicine and Pathology and Departments of Pediatrics, Medicine, and Molecular Biology...

書誌事項

公開日
1999-03-30
DOI
  • 10.1073/pnas.96.7.3819
公開者
Proceedings of the National Academy of Sciences

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説明

<jats:p>Dissecting aortic aneurysm is the hallmark of Marfan syndrome (MFS) and the result of mutations in fibrillin-1, the major constituent of elastin-associated extracellular microfibrils. It is yet to be established whether dysfunction of fibrillin-1 perturbs the ability of the elastic vessel wall to sustain hemodynamic stress by disrupting microfibrillar assembly, by impairing the homeostasis of established elastic fibers, or by a combination of both mechanisms. The pathogenic sequence responsible for the mechanical collapse of the elastic lamellae in the aortic wall is also unknown. Targeted mutation of the mouse fibrillin-1 gene has recently suggested that deficiency of fibrillin-1 reduces tissue homeostasis rather than elastic fiber formation. Here we describe another gene-targeting mutation, mgR, which shows that underexpression of fibrillin-1 similarly leads to MFS-like manifestations. Histopathological analysis of mgR/mgR specimens implicates medial calcification, the inflammatory–fibroproliferative response, and inflammation-mediated elastolysis in the natural history of dissecting aneurysm. More generally, the phenotypic severity associated with various combinations of normal and mutant fibrillin-1 alleles suggests a threshold phenomenon for the functional collapse of the vessel wall that is based on the level and the integrity of microfibrils.</jats:p>

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