Impaired proteoglycan glycosylation, elevated TGF-β signaling, and abnormal osteoblast differentiation as the basis for bone fragility in a mouse model for gerodermia osteodysplastica

Gerodermia osteodysplastica (GO) is characterized by skin laxity and early-onset osteoporosis. GORAB, the responsible disease gene, encodes a small Golgi protein of poorly characterized function. To circumvent neonatal lethality of the Gorab(Null) full knockout, Gorab was conditionally inactivated i...

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Detalles Bibliográficos
Autores principales: Chan, Wing Lee, Steiner, Magdalena, Witkos, Tomasz, Egerer, Johannes, Busse, Björn, Mizumoto, Shuji, Pestka, Jan M., Zhang, Haikuo, Hausser, Ingrid, Khayal, Layal Abo, Ott, Claus-Eric, Kolanczyk, Mateusz, Willie, Bettina, Schinke, Thorsten, Paganini, Chiara, Rossi, Antonio, Sugahara, Kazuyuki, Amling, Michael, Knaus, Petra, Chan, Danny, Lowe, Martin, Mundlos, Stefan, Kornak, Uwe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2018
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5880397/
https://www.ncbi.nlm.nih.gov/pubmed/29561836
http://dx.doi.org/10.1371/journal.pgen.1007242

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