Abstract:
:Reducible compounds that participate in crosslinking were analyzed in hydroxylysine-deficient collagens of patients with a heritable disorder of connective tissue. After treatment with [(3)H]sodium borohydride, new compounds, as well as a totally different pattern of tritiated compounds, were found in hydroxylysine-deficient collagen from skin as compared with age-matched controls. The amount of desmosines detected indicated that more elastin was present in abnormal skin than in control skin.Bone collagen, which was not as deficient in hydroxylysine as skin collagen, had the same compounds as normal bone collagen, but their relative proportions were altered, consistent with a deficiency of hydroxylysine, a precursor of the crosslinks. Although the content of hydroxylysine in collagen of cartilage is essentially normal in these patients, analysis after reduction revealed a different pattern of reduced compounds from that of normal cartilage. It is speculated that Type II collagen, the major collagen component in cartilage, contains a normal amount of hydroxylysine, while Type I collagen, which is the major source of the crosslinks, is hydroxylysine-deficient. This distribution would explain the findings of an abnormal profile of reducible compounds despite an almost normal total hydroxylysine content. The finding that the deficiency of hydroxylysine in the collagen of these patients is accompanied by changes in number, chemical nature, and, probably, distribution of crosslinkages, and the previously reported alterations in the solubility characteristics, suggest that at least some skeletal and connective tissue abnormalities are directly related to underlying molecular pathology.
journal_name
Proc Natl Acad Sci U S Aauthors
Eyre DR,Glimcher MJdoi
10.1073/pnas.69.9.2594subject
Has Abstractpub_date
1972-09-01 00:00:00pages
2594-8issue
9eissn
0027-8424issn
1091-6490journal_volume
69pub_type
杂志文章abstract::Hookworms are hematophagous nematodes that infect a wide range of mammalian hosts, including humans. There has been speculation for nearly a century as to the identity of the anticoagulant substances) used by these organisms to subvert host hemostasis. Using molecular cloning, we describe a family of potent small prot...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.93.5.2149
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.87.16.6156
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.1812334116
更新日期:2019-06-25 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
pub_type: 杂志文章
doi:10.1073/pnas.120168097
更新日期:2000-07-18 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
pub_type: 杂志文章
doi:10.1073/pnas.0832472100
更新日期:2003-06-24 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
pub_type: 杂志文章
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更新日期:2013-06-25 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2013-02-19 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2007-05-22 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1994-03-29 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1991-04-15 00:00:00
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更新日期:2019-12-10 00:00:00
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更新日期:2010-01-05 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1990-05-01 00:00:00
abstract::Sands from various geographic locations reduce N(2) from the air to NH(3) and traces of N(2)H(4) on exposure to sunlight. This N(2) photofixation occurs under sterile conditions on the surface of finely dispersed titanium minerals such as rutile, utilizing reducing equivalents generated through the photolysis of chemi...
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更新日期:1983-06-01 00:00:00