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Modeling cartilage pathology in mucopolysaccharidosis VI using iPSCs reveals early dysregulation of chondrogenic and metabolic gene expression

Mucopolysaccharidosis type VI (MPS VI) is a metabolic disorder caused by disease-associated variants in the Arylsulfatase B (ARSB) gene, resulting in ARSB enzyme deficiency, lysosomal glycosaminoglycan accumulation, and cartilage and bone pathology. The molecular response to MPS VI that results in c...

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Detalles Bibliográficos
Autores principales:	Broeders, M., van Rooij, Jgj, Oussoren, E., van Gestel, Tjm, Smith, Ca, Kimber, Sj, Verdijk, Rm, Wagenmakers, Maem, van den Hout, Jmp, van der Ploeg, At, Narcisi, R., Pijnappel, Wwmp
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Frontiers Media S.A. 2022
Materias:	Bioengineering and Biotechnology
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9763729/ https://www.ncbi.nlm.nih.gov/pubmed/36561048 http://dx.doi.org/10.3389/fbioe.2022.949063

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