Article
Cathepsin-Mediated Alterations in TGFß-Related Signaling Underlie Disrupted Cartilage and Bone Maturation Associated With Impaired Lysosomal Targeting.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Mar 2016
Flanagan-Steet Heather, Aarnio Megan, Kwan Brian, Guihard Pierre, Petrey Aaron, Haskins Mark, Blanchard Frederic, Steet Richard
Abstract excerpt
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 that connect hydrolase activity to the onset of disease phenotypes remain poorly understood. Here we link...
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