Altered interaction and distribution of glycosaminoglycans and growth factors in mucopolysaccharidosis type I bone disease
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- 作者：Sandra D.K. Kingmaa ; b ; s.d.kingma@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author ; Tom Wagemansa ; b ; a.h.wagemans@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author ; Lodewijk IJlsta ; l.ijlst@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author ; Antonius L.J.J. Bronckersc ; a.bronckers@acta.nl" class="auth_mail" title="E-mail the corresponding author ; Toin H. van Kuppeveltd ; Toin.vanKuppevelt@Radboudumc.nl" class="auth_mail" title="E-mail the corresponding author ; Vincent Evertsc ; v.everts@acta.nl" class="auth_mail" title="E-mail the corresponding author ; Frits A. Wijburgb ; f.a.wijburg@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author ; Naomi van Vliesa ; b ; n.vanvlies@amc.uva.nl" class="auth_mail" title="E-mail the corresponding author
- 关键词：Mucopolysaccharidosis ; Glycosaminoglycans ; Growth factors ; Bone disease ; Fibroblast growth factor ; Indian hedgehog
- 刊名：Bone
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：88
- 期：Complete
- 页码：92-100
- 全文大小：2463 K
文摘
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MPS I chondrocytes exhibit alterations in FGF2 signaling and GAG distribution.
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MPS I mouse bones exhibit alterations in growth after FGF2 incubation and alterations in GAG and growth factor distribution.
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Altered interaction between GAGs and growth factors likely contributes to MPS bone disease.
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Growth factor therapy might be a future therapeutic strategy for MPS I bone disease.