Lack of collagen XVIII/endostatin results in eye abnormalities

Mice lacking collagen XVIII and its proteolytically derived product endostatin show delayed regression of blood vessels in the vitreous along the surface of the retina after birth and lack of or abnormal outgrowth of retinal vessels. This suggests that collagen XVIII/endostatin is critical for norma...

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Bibliographic Details
Published in:The EMBO journal 2002-04, Vol.21 (7), p.1535-1544
Main Authors: Fukai, Naomi, Eklund, Lauri, Marneros, Alexander G., Oh, Suk Paul, Keene, Douglas R., Tamarkin, Lawrence, Niemelä, Merja, Ilves, Mika, Li, En, Pihlajaniemi, Taina, Olsen, Bjorn R.
Format: Article
Language:English
Subjects:
Angiogenesis Inhibitors - metabolism
Animals
Antineoplastic Agents - metabolism
Basement Membrane - metabolism
Basement Membrane - ultrastructure
Collagen - genetics
Collagen - metabolism
Collagen - physiology
Collagen Type XVIII
collagen XVIII
Disease Models, Animal
EMBO05
EMBO24
endostatin
Endostatins
Endothelial Growth Factors - genetics
Eye
Eye - blood supply
Eye - metabolism
Eye - pathology
Eye - ultrastructure
eye abnormalities
Eye Abnormalities - metabolism
Eye Abnormalities - pathology
Female
Fibrosarcoma
Gene Expression
immunogold labeling
Knobloch syndrome
knockout mice
Lymphokines - genetics
Male
Melanoma
Membranes
Mice
Mice, Inbred C57BL
Mice, Knockout
Peptide Fragments - genetics
Peptide Fragments - metabolism
Peptide Fragments - physiology
Retinal Vessels - growth & development
Tissue Distribution
Vascular Endothelial Growth Factor A
Vascular Endothelial Growth Factors
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Summary:Mice lacking collagen XVIII and its proteolytically derived product endostatin show delayed regression of blood vessels in the vitreous along the surface of the retina after birth 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 immunogold labeling with antibodies against collagen XVIII. Labeling at sites where collagen fibrils in the vitreous are connected with the inner limiting membrane and separation of the vitreal matrix from the inner limiting membrane in 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.
ISSN:0261-4189
1460-2075
1460-2075
DOI:10.1093/emboj/21.7.1535