| 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. | |
| | |
MedLine Citation:
|
PMID: 19687302 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Dermatan sulfate epimerase 1 (DS-epi1) and DS-epi2 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 in 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 their 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 collagen fibril formation and reduce the tensile strength of DS-epi1-null skin. |
| | |
Authors:
|
Marco Maccarana; Sebastian Kalamajski; Mads Kongsgaard; S Peter Magnusson; Ake Oldberg; Anders Malmström |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-08-17 |
Journal Detail:
|
Title: Molecular and cellular biology Volume: 29 ISSN: 1098-5549 ISO Abbreviation: Mol. Cell. Biol. Publication Date: 2009 Oct |
Date Detail:
|
Created Date: 2009-09-29 Completed Date: 2009-11-03 Revised Date: 2010-09-27 |
Medline Journal Info:
|
Nlm Unique ID: 8109087 Medline TA: Mol Cell Biol Country: United States |
Other Details:
|
Languages: eng Pagination: 5517-28 Citation Subset: IM |
Affiliation:
|
Department of Experimental Medical Science, Biomedical Center D12, Lund University, SE-221 84 Lund, Sweden. marco.maccarana@med.lu.se |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Animals Carbohydrate Epimerases / genetics, metabolism* Collagen / metabolism*, ultrastructure Dermatan Sulfate / genetics, metabolism* Extracellular Matrix Proteins / metabolism Iduronic Acid / metabolism* Mice Mice, Inbred C57BL Mice, Knockout Microscopy, Electron, Transmission Proteoglycans / metabolism Skin / enzymology, metabolism*, ultrastructure |
| Chemical | |
Reg. No./Substance:
|
0/Extracellular Matrix Proteins; 0/Proteoglycans; 0/decorin; 24967-94-0/Dermatan Sulfate; 3402-98-0/Iduronic Acid; 9007-34-5/Collagen; EC 5.1.3.-/Carbohydrate Epimerases; EC 5.1.3.19/dermatan sulfate epimerase 1, mouse |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Hyperactivated NF-{kappa}B and AP-1 transcription factors promote highly accessible chromatin and co...
Next Document: Generation of a novel allelic series of cryptochrome mutants via mutagenesis reveals residues involv...