Aberrant Expressions of Decorin and Biglycan Genes in the Carbohydrate-Deficient Glycoprotein Syndrome

• GU Jianguo

  Department of Molecular Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health

• WADA Yoshinao

  Department of Molecular Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health

抄録

Carbohydrate-deficient glycoprotein syndrome (CDGS) is a congenital disorder characterized by neurological and developmental defects. We have examined the expressions of the small proteoglycans decorin and biglycan in cultured skin fibroblasts from a patient with CDGS Type-I. Northern blotting analysis identified a marked reduction in decorin mRNA and an increase in biglycan mRNA levels. The decorin protein in the culture medium was decreased. Responses to interleukin-1 β (IL-1 β) and transforming growth factor-β 1 (TGF-β 1) were apparently abnormal; decorin was only slightly up-regulated by IL-1 β, while biglycan was markedly down-regulated by IL-1 β and significantly up-regulated by TGF-β 1. The constitutional and developmental abnormalities characteristic of CDGS may be associated with such derangements in the expression of proteoglycan genes.

収録刊行物

• The journal of biochemistry

  The journal of biochemistry 117 (6), 1276-1279, 1995-06-01

  社団法人 日本生化学会

キーワード

• biglycan
• CDGS
• decorin
• expression
• fibroblast

詳細情報

• CRID: 1570009749448041472
• NII論文ID: 10005182041
• NII書誌ID: AA00694073
• ISSN: 0021924X
• Web Site: https://jlc.jst.go.jp/DN/JALC/00081575695?from=CiNii
• Web Site: https://search.jamas.or.jp/link/ui/1996016194
• 本文言語コード: en
• データソース種別:
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