Deficient Expression of Decorin in Infantile Progeroid Patients

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Deficient expression of decorin in infantile progeroid patients.

Fibroblasts from young patients exhibiting clinical features of progeroidal syndromes showed decreased biosynthesis of the small proteoglycan decorin. Cells in culture were metabolically labeled, and proteoglycans secreted into the medium were analyzed electrophoretically after immunoprecipitation with antibodies raised against decorin and biglycan. Fluorograms showed regularly a reduction to 1...

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Decorin Affects Cytokine Expression

The proteoglycan decorin modulates leukocyte recruitment during delayed-type hypersensitivity (DTH) responses. Decorindeficient (Dcn) mice show reduced edema formation during the first 24 h with a concurrent attenuated recruitment of Cd8 leukocytes in the inflamed Dcn ears. The aim of this study was to elucidate the molecular pathways affected by the loss of decorin. In vivo, reduced numbers of...

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Deficient expression of the gene coding for decorin in a lethal form of Marfan syndrome.

The markedly decreased level of the messenger RNA of decorin, an abundant dermatan/chondroitin sulfate proteoglycan was found in the skin fibroblast culture of a lethally sick Marfan infant. Also, the amount of decorin polypeptide in the culture medium of the fibroblasts of this infant was markedly decreased. When the effect of interleukin-1 beta on the transcription of decorin was tested in th...

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Alterations in lung mechanics in decorin-deficient mice.

Decorin, a small leucine-rich proteoglycan with a widespread tissue distribution, is required for the normal fibrillogenesis of collagen in most tissues. Because collagen is important in determining the elastic behavior of the lung, we hypothesized that lung tissue mechanics would be altered in a mutant mouse in which the single decorin gene was abrogated by targeted deletion (Dcn-/-). Complex ...

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ژورنال

عنوان ژورنال: Trends in Glycoscience and Glycotechnology

سال: 1993

ISSN: 0915-7352,1883-2113

DOI: 10.4052/tigg.5.482