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Transforming growth factor-β signaling pathway in Marfan's syndrome: a preliminary histopathological study.
- Source :
-
VASA. Zeitschrift fur Gefasskrankheiten [Vasa] 2011 Sep; Vol. 40 (5), pp. 369-74. - Publication Year :
- 2011
-
Abstract
- Background: Marfan's syndrome is an inherited disorder that affects the connective tissue. It has been proposed that mutations of FBN1 gene or of transforming growth factor (TGF)-beta type II receptor may be responsible for its pathogenesis. However, the role of TGF-beta signaling pathway in the development of Marfan's syndrome has not been comprehensively investigated.<br />Materials and Methods: Surgical specimens of the aorta were obtained from two female Marfan patients, and the control aortic tissue was taken from an autopsy of a healthy individual. The aortic specimens were examined with hematoxylin-eosin, Masson's trichrome, von Gieson/victoria blue-van Gieson bichrome, and immunohistochemical stainings of TGF-beta1, TGF-beta type I receptor, Smad2/3, Smad4 and Smad7.<br />Results: Hematoxylin-eosin staining demonstrated severe elastic lamellar disruption and patchy vascular smooth muscle dissolution in the aortic media of the Marfan patients. Collagen deposition, interlamilar elastic fiber fragmentation, loss or proliferation, and acid mucopolysaccharide accumulation were observed in the disarrayed aortic wall structures of Marfan patients by Masson's trichrome, victoria blue-van Gieson bichrome, and Alcian blue and periodic schiff's (AB-PAS) stainings, respectively. By immunohistochemistry, structural disruptions with enhanced TGF-beta;1 in the cytoplasm, Smad2/3 in the interstices, Smad4 in the cytoplasm, nuclei or interstices, and OOO Smad7, in the nucleus along with attenuated TGF-beta type I receptor in the aortic tissues of Marfan patients in comparison to the healthy control.<br />Conclusions: Marfan patients may have aberrant TGF-beta signaling pathway associated with increased collagen deposition, interlamilar elastic fiber degenerative changes, and acid mucopolysaccharide accumulation. The signaling dysregulation may play an important role in the pathogenesis of this genetic disorder.
- Subjects :
- Aorta pathology
Case-Control Studies
Collagen analysis
Elastic Tissue pathology
Female
Glycosaminoglycans analysis
Humans
Immunohistochemistry
Marfan Syndrome pathology
Receptor, Transforming Growth Factor-beta Type I
Smad2 Protein analysis
Smad3 Protein analysis
Smad4 Protein analysis
Smad7 Protein analysis
Staining and Labeling
Aorta chemistry
Marfan Syndrome metabolism
Protein Serine-Threonine Kinases analysis
Receptors, Transforming Growth Factor beta analysis
Signal Transduction
Transforming Growth Factor beta1 analysis
Subjects
Details
- Language :
- English
- ISSN :
- 0301-1526
- Volume :
- 40
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- VASA. Zeitschrift fur Gefasskrankheiten
- Publication Type :
- Academic Journal
- Accession number :
- 21948779
- Full Text :
- https://doi.org/10.1024/0301-1526/a000133