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Dermatan Sulfate Epimerase 1-Deficient Mice have Reduced Content and Changed Distribution of Iduronic acids in Dermatan Sulfate and an Altered Collagen Structure in Skin.

Maccarana, Marco LU ; Kalamajski, Sebastian LU ; Kongsgaard, Mads; Magnusson, S Peter; Oldberg, Åke LU and Malmström, Anders LU (2009) In Molecular and Cellular Biology 29. p.5517-5528
Abstract
Dermatan sulfate epimerase 1 (DS-epi1) and 2 convert glucuronic acid to iduronic acid in chondroitin/dermatan sulfate biosynthesis. Here we report on the generation of DS-epi1-null mice and the resulting alterations in the chondroitin/dermatan polysaccharide chains. The numbers of long blocks of adjacent iduronic acids are greatly decreased in skin decorin and biglycan chondroitin/dermatan sulfate, along with a parallel decrease of iduronic-2-O-sulfated-galactosamine-4-O-sulfated structures. Both iduronic acid blocks and iduronic acids surrounded by glucuronic acids are also decreased in versican-derived chains. DS-epi1-deficient mice are smaller than wild-type littermates, but otherwise have no gross macroscopic alterations. The lack of... (More)
Dermatan sulfate epimerase 1 (DS-epi1) and 2 convert glucuronic acid to iduronic acid in chondroitin/dermatan sulfate biosynthesis. Here we report on the generation of DS-epi1-null mice and the resulting alterations in the chondroitin/dermatan polysaccharide chains. The numbers of long blocks of adjacent iduronic acids are greatly decreased in skin decorin and biglycan chondroitin/dermatan sulfate, along with a parallel decrease of iduronic-2-O-sulfated-galactosamine-4-O-sulfated structures. Both iduronic acid blocks and iduronic acids surrounded by glucuronic acids are also decreased in versican-derived chains. DS-epi1-deficient mice are smaller than wild-type littermates, but otherwise have no gross macroscopic alterations. The lack of DS-epi1 affects the chondroitin/dermatan sulfate in many proteoglycans and the consequences for skin collagen structure were initially analyzed. We found that the skin collagen architecture was altered, and electron microscopy showed that the DS-epi1-null fibrils have a larger diameter than the wild-type fibrils. The altered chondroitin/dermatan sulfate chains carried by decorin in skin are likely to affect the collagen fibril formation and reduce the tensile strength of DS-epi1-null skin. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Molecular and Cellular Biology
volume
29
pages
5517 - 5528
publisher
American Society for Microbiology
external identifiers
  • wos:000270271100011
  • pmid:19687302
  • scopus:70449112926
ISSN
0270-7306
DOI
10.1128/MCB.00430-09
language
English
LU publication?
yes
id
017b6511-fdc6-449e-8f4b-a0e9047db663 (old id 1469640)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19687302?dopt=Abstract
date added to LUP
2009-09-07 14:30:50
date last changed
2017-09-24 04:38:03
@article{017b6511-fdc6-449e-8f4b-a0e9047db663,
  abstract     = {Dermatan sulfate epimerase 1 (DS-epi1) and 2 convert glucuronic acid to iduronic acid in chondroitin/dermatan sulfate biosynthesis. Here we report on the generation of DS-epi1-null mice and the resulting alterations in the chondroitin/dermatan polysaccharide chains. The numbers of long blocks of adjacent iduronic acids are greatly decreased in skin decorin and biglycan chondroitin/dermatan sulfate, along with a parallel decrease of iduronic-2-O-sulfated-galactosamine-4-O-sulfated structures. Both iduronic acid blocks and iduronic acids surrounded by glucuronic acids are also decreased in versican-derived chains. DS-epi1-deficient mice are smaller than wild-type littermates, but otherwise have no gross macroscopic alterations. The lack of DS-epi1 affects the chondroitin/dermatan sulfate in many proteoglycans and the consequences for skin collagen structure were initially analyzed. We found that the skin collagen architecture was altered, and electron microscopy showed that the DS-epi1-null fibrils have a larger diameter than the wild-type fibrils. The altered chondroitin/dermatan sulfate chains carried by decorin in skin are likely to affect the collagen fibril formation and reduce the tensile strength of DS-epi1-null skin.},
  author       = {Maccarana, Marco and Kalamajski, Sebastian and Kongsgaard, Mads and Magnusson, S Peter and Oldberg, Åke and Malmström, Anders},
  issn         = {0270-7306},
  language     = {eng},
  pages        = {5517--5528},
  publisher    = {American Society for Microbiology},
  series       = {Molecular and Cellular Biology},
  title        = {Dermatan Sulfate Epimerase 1-Deficient Mice have Reduced Content and Changed Distribution of Iduronic acids in Dermatan Sulfate and an Altered Collagen Structure in Skin.},
  url          = {http://dx.doi.org/10.1128/MCB.00430-09},
  volume       = {29},
  year         = {2009},
}