LACK OF COLLAGEN XVIII/ENDOSTATIN RESULTS IN EYE ABNORMALITIES

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Coll 8a1, encoding α1 (XVIII) collagen, was targeted by homologous recombination with a PGK-neo inserted into exon 30 of the gene and collagen XVIII null mice were generated. Mice lacking collagen XVIII and its proteolytically derived product endostatin show delayed regression of hyaloid vessels in the vitreous along the surface of the retina after birth, decreased upregulation of VEGF in retinal neuroglial cells and lack of or abnormal outgrowth of retinal vessels. This suggests that collagen XVIII/endostatin is critical for normal blood vessel formation in the eye. All basement membranes in wild type eyes, except Descemet's membrane, showed immumogold-labeling with antibodies against collagen XVIII. Strong labeling at sites where collagen fibrils inserted into the inner limiting membrane in wild type mice and separation between fibrils and the inner limiting membrane of mutant mice indicate that collagen XVIII is important for anchoring vitreal collagen fibrils to the inner limiting membrane. The findings provide an explanation for high myopia, vitreoretinal degeneration and retinal detachment seen in patients with Knobloch syndrome caused by loss of function mutations in collagen XVIII.
日本結合組織学会の論文