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Bone samples from three patients with type I, nine patients with type III, and eight patients with type IV OI were analyzed and compared with human bone from nine controls. The concentration of the two chemical forms of the mature collagen cross-linking amino acids, hydroxylysylpyridinoline (HP) and lysylpyridinoline (LP), and the ratio HP\/LP were found to be alike in bone collagen of OI patients and healthy controls. However, the c-axis crystallinity of the apatite was found to be reduced in the type III and IV OI patients compared with controls. Regression analysis of crosslink concentrations and c-axis crystallinity in OI bones did not show any correlation. Therefore, collagen molecules deposited in the extracellular matrix of OI bone appear to fulfill the structural requirements for the action of the enzyme lysyl oxidase, such that a normal concentration of intermolecular crosslinks is formed compared with healthy bone. Consequently, crosslink formation and apatite crystal growth seem to be regulated independently in OI bone.<\/jats:p>","DOI":"10.1002\/jbmr.5650080203","type":"journal-article","created":{"date-parts":[[2010,2,3]],"date-time":"2010-02-03T16:46:30Z","timestamp":1265215590000},"page":"133-137","source":"Crossref","is-referenced-by-count":41,"title":["Collagen crosslinks and mineral crystallinity in bone of patients with osteogenesis imperfecta"],"prefix":"10.1093","volume":"8","author":[{"given":"Ulrich","family":"Vetter","sequence":"first","affiliation":[{"name":"Bone Research Branch, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland"},{"name":"Johann Wolfgang Goethe Universit\u00e4t, Kinderklinik, Frankfurt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Ann","family":"Weis","sequence":"additional","affiliation":[{"name":"Departments of Orthopaedics and Biochemistry, University of Washington, Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"M\u00f6rike","sequence":"additional","affiliation":[{"name":"Bindegewebslabor der Universit\u00e4tskinkerklinik, Ulm, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward David","family":"Eanes","sequence":"additional","affiliation":[{"name":"Bone Research Branch, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David Rodney","family":"Eyre","sequence":"additional","affiliation":[{"name":"Departments of Orthopaedics and Biochemistry, University of Washington, Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[1993,2,1]]},"reference":[{"key":"2024013113121466900_bib1","volume-title":"Heritable Disorders of Connective Tissue","author":"McKusick","year":"1972"},{"key":"2024013113121466900_bib2","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1002\/ajmg.1320340114","article-title":"Inherited disorders of collagen gene structure and expression","volume":"34","author":"Byers","year":"1985","journal-title":"Am J Med Genet"},{"key":"2024013113121466900_bib3","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1038\/348018a0","article-title":"Bone disease cracks genetics","volume":"348","author":"Sykes","year":"1990","journal-title":"Nature"},{"key":"2024013113121466900_bib4","doi-asserted-by":"crossref","first-page":"2870","DOI":"10.1073\/pnas.82.9.2870","article-title":"Intron-mediated recombination may cause a deletion in an \u03b11 type I collagen chain in a lethal form of osteogenesis imperfecta","volume":"82","author":"Barsh","year":"1985","journal-title":"Proc Natl Acad Sci USA"},{"key":"2024013113121466900_bib5","first-page":"685","article-title":"Diminished type I collagen synthesis and reduced alpha 1(I) collagen messenger RNA in cultured fibroblasts from patients with doiminantly inherited type I osteogenesis imperfecta","volume":"71","author":"Rowe","year":"1985","journal-title":"J Clin Invest"},{"key":"2024013113121466900_bib6","first-page":"237","article-title":"Perinatal lethal osteogenesis imperfecta (OI type II). 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