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© The Rockefeller University Press, 0021-9525/1998//1277 $5.00
The Journal of Cell Biology, Volume 141, Number 5, , 1998 1277-1286


Articles

Lumican Regulates Collagen Fibril Assembly: Skin Fragility and Corneal Opacity in the Absence of Lumican



Shukti Chakravarti*, Terry Magnuson{ddagger}, Jonathan H. Lass§, Karl J. Jepsen||, Christian LaMantia{ddagger}, and Heidi Carroll*

* Department of Medicine and Genetics, {ddagger} Department of Genetics, § Department of Ophthalmology, and || Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952

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.


Abbreviations used in this paper: EDS, Ehlers-Danos syndrome; ES, embryonic stem; GAG, glycosaminoglycan; KS, keratan sulfate; LRP, leucine-rich proteoglycan family.

Address all correspondence to Shukti Chakravarti, Ph.D., Departments of Medicine and Genetics, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Office: 423 BRB, Cleveland, OH 44106-4952. Tel.: (216) 368-1828. Fax: (216) 368-1674. E-mail: sxc76{at}po.cwru.edu



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