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  Ext1-dependent heparan sulfate regulates the range of Ihh signaling during endochondral ossification

Koziel, L., Kunath, M., Kelly, O. G., & Vortkamp, A. (2004). Ext1-dependent heparan sulfate regulates the range of Ihh signaling during endochondral ossification. Developmental Cell, 6(6), 801-813. doi:10.1016/j.devcel.2004.05.009.

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Genre: Journal Article
Alternative Title : Dev Cell

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 Creators:
Koziel, Lydia1, Author
Kunath, Melanie1, Author
Kelly, Olivia G., Author
Vortkamp, Andrea2, Author              
Affiliations:
1Max Planck Society, ou_persistent13              
2Independent Junior Research Groups (OWL), Max Planck Institute for Molecular Genetics, Max Planck Society, ou_1433554              

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 Abstract: Exostosin1 (Ext1) belongs to a family of glycosyltransferases necessary for the synthesis of the heparan sulfate (HS) chains of proteoglycans, which regulate signaling of several growth factors. Loss of tout velu (ttv), the homolog of Ext1 in Drosophila, inhibits Hedgehog movement. In contrast, we show that reduced HS synthesis in mice carrying a hypomorphic mutation in Ext1 results in an elevated range of Indian hedgehog (Ihh) signaling during embryonic chondrocyte differentiation. Our data suggest a dual function for HS: First, HS is necessary to bind Hedgehog in the extracellular space. Second, HS negatively regulates the range of Hedgehog signaling in a concentration-dependent manner. Additionally, our data indicate that Ihh acts as a long-range morphogen, directly activating the expression of parathyroid hormone-like hormone. Finally, we propose that the development of exostoses in the human Hereditary Multiple Exostoses syndrome can be attributed to activation of Ihh signaling.

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Language(s): eng - English
 Dates: 2004-06-07
 Publication Status: Published in print
 Pages: -
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 Table of Contents: -
 Rev. Type: -
 Identifiers: eDoc: 228632
DOI: 10.1016/j.devcel.2004.05.009
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Title: Developmental Cell
  Alternative Title : Dev Cell
Source Genre: Journal
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Pages: - Volume / Issue: 6 (6) Sequence Number: - Start / End Page: 801 - 813 Identifier: ISSN: 1534-5807