Lumican Regulates Collagen Fibril Assembly: Skin Fragility and Corneal Opacity in the Absence of Lumican
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- Shukti Chakravarti
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
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- Terry Magnuson
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
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- Jonathan H. Lass
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
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- Karl J. Jepsen
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
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- Christian LaMantia
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
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- Heidi Carroll
- *Department of Medicine and Genetics, ‡Department of Genetics, §Department of Ophthalmology, and ‖Department of Orthopaedics, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106-4952
抄録
<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>
収録刊行物
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- The Journal of Cell Biology
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The Journal of Cell Biology 141 (5), 1277-1286, 1998-06-01
Rockefeller University Press
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詳細情報
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- CRID
- 1363388843655721216
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- NII論文ID
- 80010381580
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- ISSN
- 15408140
- 00219525
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