Document Detail

Collagen studies in congenital cutis laxa.
MedLine Citation:
PMID:  3632035     Owner:  NLM     Status:  MEDLINE    
A case of congenital cutis laxa (CCL) of unclear mode of inheritance associated with multiple pulmonary artery branch stenosis was extensively investigated to assess possible correlations between clinical, ultrastructural, and biochemical features. Light microscopy revealed that elastic fibers were absent in the papillary dermis, while hypoplastic elsewhere. Transmission electron microscopy showed a poor elastin matrix content in some elastic fibers, variable diameters of collagen fibrils, and abundant glycogen granules in most dermal cells. Measurement of collagen fibril diameters, using an image analyzer, was carried out in the patient and two age- and site-matched controls. A bimodal distribution was found in the upper reticular dermis of the patient. In vitro analysis of collagen in skin fibroblast cultures of the patient showed increased collagen synthesis with a balanced production of type I and type III procollagens. Our study confirms that CCL represents a disorder both of collagen and elastic connective tissue.
A Taïeb; M Aumailley; D Courouge-Dorcier; M Rabaud; P Bioulac-Sage; J E Surlève-Bazeille; J Maleville
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of dermatological research     Volume:  279     ISSN:  0340-3696     ISO Abbreviation:  Arch. Dermatol. Res.     Publication Date:  1987  
Date Detail:
Created Date:  1987-10-22     Completed Date:  1987-10-22     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8000462     Medline TA:  Arch Dermatol Res     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  308-14     Citation Subset:  IM    
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MeSH Terms
Cells, Cultured
Collagen / classification,  metabolism*
Cutis Laxa / congenital,  metabolism*,  pathology
Microscopy, Electron
Skin / metabolism
Reg. No./Substance:

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