{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T16:26:27Z","timestamp":1792599987973,"version":"4.5.0"},"reference-count":47,"publisher":"Rockefeller University Press","issue":"5","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1998,6,1]]},"abstract":"<jats:p>Lumican, a prototypic leucine-rich proteoglycan with keratan sulfate side chains, is a major component of the cornea, dermal, and muscle connective tissues. Mice homozygous for a null mutation in lumican display skin laxity and fragility resembling certain types of Ehlers-Danlos syndrome. In addition, the mutant mice develop bilateral corneal opacification. The underlying connective tissue defect in the homozygous mutants is deregulated growth of collagen fibrils with a significant proportion of abnormally thick collagen fibrils in the skin and cornea as indicated by transmission electron microscopy. A highly organized and regularly spaced collagen fibril matrix typical of the normal cornea is also missing in these mutant mice. This study establishes a crucial role for lumican in the regulation of collagen assembly into fibrils in various connective tissues. Most importantly, these results provide a definitive link between a necessity for lumican in the development of a highly organized collagenous matrix and corneal transparency.<\/jats:p>","DOI":"10.1083\/jcb.141.5.1277","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T12:47:50Z","timestamp":1027687670000},"page":"1277-1286","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":627,"title":["Lumican Regulates Collagen Fibril Assembly: Skin Fragility and Corneal Opacity in the Absence of Lumican"],"prefix":"10.1083","volume":"141","author":[{"given":"Shukti","family":"Chakravarti","sequence":"first","affiliation":[{"name":"*Department of Medicine and Genetics, \u2021Department of Genetics, \u00a7Department of Ophthalmology, and \u2016Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 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