| Fibrillin and other matrix proteins in mitral valve prolapse syndrome. | |
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MedLine Citation:
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PMID: 14759433 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Unlike myxomatous degeneration in Marfan syndrome, which has been reported to result from a mutation in the gene that codes for the extracellular structural protein fibrillin, no specific molecular abnormality has been documented to be the underlying cause of myxomatous degeneration in mitral valve prolapse syndrome (MVPS). The present study examined the distribution of fibrillin and other extracellular matrix proteins in patients with isolated MVPS. METHODS: Mitral valve leaflets from 7 MVPS patients and 5 rheumatic heart disease (RHD) patients were characterized immunohistochemically for fibrillin, elastin, collagen I, and collagen III distribution, and compared with five normal mitral valves. RESULTS: In normal mitral valve leaflets immunostaining for fibrillin, elastin, collagen I, and collagen III revealed a fibrillary and laminar pattern in the atrialis and the spongiosa. In addition, both the collagens were present in the ventricularis, and the coarse bundles in the fibrosa exhibited alternating bandlike collagen I immunoreactivity. The staining patterns of fibrillin, elastin, and collagens I and III revealed distinctly different distribution in MVPS relative to the normal and RHD leaflets. MVPS leaflets in areas of myxoid degeneration displayed a more diffuse, weaker, and nonlaminar pattern of staining for fibrillin. Similar, but less severe abnormality of elastin, collagen I, and collagen III was also observed. Unlike diffuse abnormality in MVPS, the disruption of extracellular proteins in RHD only occurred at the site of the inflammatory damage, but the overall architecture was preserved. CONCLUSIONS: The results of the current study suggest a primary role for abnormal fibrillin and other matrix proteins in producing myxoid degeneration of mitral valve leaflets in MVPS. |
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Authors:
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Joseph F Nasuti; Paul J Zhang; Michael D Feldman; Terri Pasha; Jasvir S Khurana; Joseph H Gorman; Robert C Gorman; Jagat Narula; Navneet Narula |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Annals of thoracic surgery Volume: 77 ISSN: 0003-4975 ISO Abbreviation: Ann. Thorac. Surg. Publication Date: 2004 Feb |
Date Detail:
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Created Date: 2004-02-04 Completed Date: 2004-03-17 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 15030100R Medline TA: Ann Thorac Surg Country: United States |
Other Details:
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Languages: eng Pagination: 532-6 Citation Subset: AIM; IM |
Affiliation:
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Hospital of University of Pennsylvania, Philadelphia, PA, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Collagen Type I / analysis Collagen Type III / analysis Elastin / analysis Extracellular Matrix / pathology Extracellular Matrix Proteins / analysis* Female Humans Immunoenzyme Techniques Male Microfilament Proteins / analysis* Middle Aged Mitral Valve / pathology Mitral Valve Prolapse / pathology* Reference Values Rheumatic Heart Disease / pathology |
| Chemical | |
Reg. No./Substance:
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0/Collagen Type I; 0/Collagen Type III; 0/Extracellular Matrix Proteins; 0/Microfilament Proteins; 0/fibrillin; 9007-58-3/Elastin |
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