Cathepsin-Mediated Alterations in TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting

Hypersecretion of acid hydrolases is a hallmark feature of mucolipidosis II (MLII), a lysosomal storage disease caused by loss of carbohydrate-dependent lysosomal targeting. Inappropriate extracellular action of these hydrolases is proposed to contribute to skeletal pathogenesis, but the mechanisms...

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Detalhes bibliográficos
Publicado no:J Bone Miner Res
Main Authors: Flanagan-Steet, Heather, Aarnio, Megan, Kwan, Brian, Guihard, Pierre, Petrey, Aaron, Haskins, Mark, Blanchard, Frederic, Steet, Richard
Formato: Artigo
Idioma:Inglês
Publicado em: 2015
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4808492/
https://ncbi.nlm.nih.gov/pubmed/26404503
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jbmr.2722
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