|
|
|
|||||||
|
|||||||||
J. Biol. Chem., Vol. 276, Issue 43, 39788-39796, October 26, 2001
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
From the The content and fine structure
of keratan and chondroitin/dermatan sulfate in normal human corneas and
corneas affected by macular corneal dystrophies (MCD) types I and II
were examined by fluorophore-assisted carbohydrate electrophoresis.
Normal tissues (n = 11) contained 15 µg of keratan
sulfate and 8 µg of chondroitin/dermatan sulfate per mg dry weight.
Keratan sulfates consisted of ~4% unsulfated, 42% monosulfated, and
54% disulfated disaccharides with number of average chain lengths of
~14 disaccharides. Chondroitin/dermatan sulfates were significantly
longer, ~40 disaccharides per chain, and consisted of ~64%
unsulfated, 28% 4-sulfated, and 8% 6-sulfated disaccharides. The fine
structural parameters were altered in all diseased tissues. Keratan
sulfate chain size was reduced to 3-4 disaccharides; chain sulfation
was absent in MCD type I corneas and cartilages, and sulfation of both
GlcNAc and Gal was significantly reduced in MCD type II.
Chondroitin/dermatan sulfate chain sizes were also decreased in all
diseased corneas to ~15 disaccharides, and the contents of 4- and
6-sulfated disaccharides were proportionally increased. Tissue
concentrations (nanomole of chains per mg dry weight) of all
glycosaminoglycan types were affected in the disease types. Keratan
sulfate chain concentrations were reduced by ~24 and ~75% in type
I corneas and cartilages, respectively, and by ~50% in type II
corneas. Conversely, chondroitin/dermatan sulfate chain concentrations
were increased by 60-70% in types I and II corneas. Such changes
imply a modified tissue content of individual proteoglycans and/or an
altered efficiency of chain substitution on the core proteins. Together
with the finding that hyaluronan, not normally present in healthy adult
corneas, was also detected in both disease subtypes, the data support
the conclusion that a wide range of keratocyte-specific proteoglycan
and glycosaminoglycan remodeling processes are activated during
degeneration of the stromal matrix in the macular corneal dystrophies.
Altered Fine Structures of Corneal and Skeletal Keratan Sulfate
and Chondroitin/Dermatan Sulfate in Macular Corneal Dystrophy*
§,,
,,, and Shriners Hospital for Children and
Department of Biochemistry and Molecular Biology, University of South
Florida College of Medicine, Tampa, Florida 33612, the
¶ Department of Biochemistry, Rush Medical College,
Chicago, Illinois 60612, the Department of Ophthalmology, Duke
University Medical Center, Durham, North Carolina 27710, and the
** Department of Biomedical Engineering, The Lerner
Institute, the Cleveland Clinic Foundation, Cleveland, Ohio 44195
*
This work was supported by a grant from the Shriners
Hospital for Children (to A. H. P.).The costs of publication of this article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
Copyright © 2001 by The American Society for Biochemistry and Molecular Biology, Inc.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  Y. Hayashida, T. O. Akama, N. Beecher, P. Lewis, R. D. Young, K. M. Meek, B. Kerr, C. E. Hughes, B. Caterson, A. Tanigami, et al. Matrix morphogenesis in cornea is mediated by the modification of keratan sulfate by GlcNAc 6-O-sulfotransferase PNAS, September 5, 2006; 103(36): 13333 - 13338. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. Rodriguez, S. K. Roland, A. Plaas, and P. J. Roughley The Glycosaminoglycan Attachment Regions of Human Aggrecan J. Biol. Chem., July 7, 2006; 281(27): 18444 - 18450. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. E. Monzon, S. M. Casalino-Matsuda, and R. M. Forteza Identification of Glycosaminoglycans in Human Airway Secretions Am. J. Respir. Cell Mol. Biol., February 1, 2006; 34(2): 135 - 141. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Zhang, A. H. Conrad, E. S. Tasheva, K. An, L. M. Corpuz, Y. Kariya, K. Suzuki, and G. W. Conrad Detection and Quantification of Sulfated Disaccharides from Keratan Sulfate and Chondroitin/Dermatan Sulfate during Chick Corneal Development by ESI-MS/MS Invest. Ophthalmol. Vis. Sci., May 1, 2005; 46(5): 1604 - 1614. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Forlino, R. Piazza, C. Tiveron, S. D. Torre, L. Tatangelo, L. Bonafe, B. Gualeni, A. Romano, F. Pecora, A. Superti-Furga, et al. A diastrophic dysplasia sulfate transporter (SLC26A2) mutant mouse: morphological and biochemical characterization of the resulting chondrodysplasia phenotype Hum. Mol. Genet., March 15, 2005; 14(6): 859 - 871. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. L. Funderburgh, M. M. Mann, and M. L. Funderburgh Keratocyte Phenotype Mediates Proteoglycan Structure: A ROLE FOR FIBROBLASTS IN CORNEAL FIBROSIS J. Biol. Chem., November 14, 2003; 278(46): 45629 - 45637. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
N. Iida-Hasegawa, A. Furuhata, H. Hayatsu, A. Murakami, K. Fujiki, K. Nakayasu, and A. Kanai Mutations in the CHST6 Gene in Patients with Macular Corneal Dystrophy: Immunohistochemical Evidence of Heterogeneity Invest. Ophthalmol. Vis. Sci., August 1, 2003; 44(8): 3272 - 3277. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Niel, P. Ellies, P. Dighiero, J. Soria, C. Sabbagh, C. San, G. Renard, M. Delpech, and S. Valleix Truncating Mutations in the Carbohydrate Sulfotransferase 6 Gene (CHST6) Result in Macular Corneal Dystrophy Invest. Ophthalmol. Vis. Sci., July 1, 2003; 44(7): 2949 - 2953. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Yamada, Y. Okada, M. Ueno, S. Iwata, S. S. Deepa, S. Nishimura, M. Fujita, I. Van Die, Y. Hirabayashi, and K. Sugahara Determination of the Glycosaminoglycan-Protein Linkage Region Oligosaccharide Structures of Proteoglycans from Drosophila melanogaster and Caenorhabditis elegans J. Biol. Chem., August 23, 2002; 277(35): 31877 - 31886. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |