Human Molecular Genetics Advance Access published online on July 14, 2004
Human Molecular Genetics, doi:10.1093/hmg/ddh213
© 2004 by Oxford University Press
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 Collagen Research Unit, Biocenter Oulu and Department of Medical Biochemistry and Molecular Biology, University of Oulu, 90014 Oulu, Finland
* To whom correspondence should be addressed. E-mail: taina.pihlajaniemi{at}oulu.fi.
Type XVIII collagen/endostatin is known to be crucial for the eye, as witnessed by severe eye defects in Knobloch syndrome patients with mutations in this collagen and in Col18a1-/- mice. We show here that in a specific C57BL background, 20% of the Col18a1-/- mice developed hydrocephalus, and dilation of the brain ventricles was observed by MRI in all of the mutant mice. Significant broadening was observed in the epithelial basement membrane (BM) of the choroid plexuses (CP), its width being 86.4±10.52 nm, compared with 61.4±6.05 nm in wild-type mice. The CP epithelial cell morphology was balloon-shaped rather than cuboidal, and the microvilli of the apical surface of the CP epithelium contained more vacuoles in the null mice than in the wild-type, as also did the CP epithelial cells, which is suggestive of alterations in cerebrospinal fluid production. Analysis of BMs elsewhere in the body revealed a broadened epidermal BM in the Col18a1-/- mice, but this did not result in any apparent functional deficiencies. Moreover, markedly broadened BMs were found in the atrioventricular valves of the heart and in the kidney tubules, while the glomerular mesangial matrix of the kidneys was expanded in the mutant mice and serum creatinine levels were elevated, indicating alterations in kidney filtration capacity. We thus suggest that type XVIII collagen is a structurally important constituent of BMs, and that its absence can result in a variety of phenotypic alterations.
Article
Structurally altered basement membranes and hydrocephalus in a type XVIII collagen deficient mouse line
2 Biocenter Oulu and Department of Pathology, University of Oulu, 90014 Oulu, Finland
3 National Bio-NMR Facility, A.I. Virtanen Institute for Molecular Sciences, University of Kuopio, 70211 Kuopio, Finland
4 Department of Morphology, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
5 Oulu University Hospital, Clinical Laboratory, 90029 OYS, Finland
6 Collagen Research Unit, Biocenter Oulu and Department of Medical Biochemistry and Molecular Biology, University of Oulu, P.O.Box 5000, 90014 Oulu, Finland
Abstract 
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  P. Dietrich, R. Shanmugasundaram, S. E, and I. Dragatsis Congenital hydrocephalus associated with abnormal subcommissural organ in mice lacking huntingtin in Wnt1 cell lineages Hum. Mol. Genet., January 1, 2009; 18(1): 142 - 150. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Brideau, M. J. Makinen, H. Elamaa, H. Tu, G. Nilsson, K. Alitalo, T. Pihlajaniemi, and R. Heljasvaara Endostatin Overexpression Inhibits Lymphangiogenesis and Lymph Node Metastasis in Mice Cancer Res., December 15, 2007; 67(24): 11528 - 11535. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. S. Kanwar, F. R. Danesh, and S. S. Chugh Contribution of Proteoglycans Towards the Integrated Functions of Renal Glomerular Capillaries: A Historical Perspective Am. J. Pathol., July 1, 2007; 171(1): 9 - 13. [Full Text] [PDF]
|
|
|
|
|
|
S. J. Harvey, G. Jarad, J. Cunningham, A. L. Rops, J. van der Vlag, J. H. Berden, M. J. Moeller, L. B. Holzman, R. W. Burgess, and J. H. Miner Disruption of Glomerular Basement Membrane Charge through Podocyte-Specific Mutation of Agrin Does Not Alter Glomerular Permselectivity Am. J. Pathol., July 1, 2007; 171(1): 139 - 152. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. W.A.M. Celie, N. W.P. Rutjes, E. D. Keuning, R. Soininen, R. Heljasvaara, T. Pihlajaniemi, A. M. Drager, S. Zweegman, F. L. Kessler, R. H.J. Beelen, et al. Subendothelial Heparan Sulfate Proteoglycans Become Major L-Selectin and Monocyte Chemoattractant Protein-1 Ligands upon Renal Ischemia/Reperfusion Am. J. Pathol., June 1, 2007; 170(6): 1865 - 1878. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. J. Harvey and J. H. Miner Breaking Down the Barrier: Evidence against a Role for Heparan Sulfate in Glomerular Permselectivity J. Am. Soc. Nephrol., March 1, 2007; 18(3): 672 - 674. [Full Text] [PDF]
|
|
|
|
 |
|
 S. Stahl, S. Gaetzner, T. D. Mueller, and U. Felbor Endostatin phenylalanines 31 and 34 define a receptor binding site Genes Cells, September 1, 2005; 10(9): 929 - 939. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. G. Marneros and B. R. Olsen Physiological role of collagen XVIII and endostatin FASEB J, May 1, 2005; 19(7): 716 - 728. [Abstract] [Full Text] [PDF]
|
|