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  • 标题:Patients with Ehlers-Danlos syndrome type IV lack type III collagen
  • 本地全文:下载
  • 作者:F M Pope ; G R Martin ; J R Lichtenstein
  • 期刊名称:Proceedings of the National Academy of Sciences
  • 印刷版ISSN:0027-8424
  • 电子版ISSN:1091-6490
  • 出版年度:1975
  • 卷号:72
  • 期号:4
  • 页码:1314-1316
  • DOI:10.1073/pnas.72.4.1314
  • 语种:English
  • 出版社:The National Academy of Sciences of the United States of America
  • 摘要:One of the genetically distinct collagens (type III) normally found in skin, aorta, and intestine is missing from the tissues of patients with the Ehlers-Danlos syndrome type IV. While skin fibroblasts from other individuals synthesize both types I and III collagen. Ehlers-Danlos syndrome IV cells synthesize only type I. These results suggest that the fragile skin, blood vessels, and intestines of Ehlers-Danlos syndrome IV patients result from an absence of type III collagen.
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