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dc.contributor.authorChazeirat, T.
dc.contributor.authorDenamur, S.
dc.contributor.authorBojarski, K.K.
dc.contributor.authorAndrault, P.-M.
dc.contributor.authorSizaret, D.
dc.contributor.authorZhang, F.
dc.contributor.authorSaidi, A.
dc.contributor.authorTardieu, M.
dc.contributor.authorLinhardt, Robert J.
dc.contributor.authorLabarthe, F.
dc.contributor.authorBrömme, D.
dc.contributor.authorSamsonov, S.A.
dc.contributor.authorLalmanach, G.
dc.contributor.authorLecaille, F.
dc.identifier.citationThe abnormal accumulation 1 of heparan sulfate in patients with mucopolysaccharidosis prevents the elastolytic activity of cathepsin V, T. Chazeirat, S. Denamur, K. K. Bojarski, P.-M. Andrault, D. Sizaret, F. Zhang, A. Saidi, M. Tardieu, R. J. Linhardt, F. Labarthe, D. Brömme, S. A. Samsonov, G. Lalmanach, F. Lecaille, Carbohydrate Polymers, 253, 117261, 2020.
dc.descriptionCarbohydrate Polymers, 253, 117261
dc.descriptionNote : if this item contains full text it may be a preprint, author manuscript, or a Gold OA copy that permits redistribution with a license such as CC BY. The final version is available through the publisher’s platform.
dc.description.abstractMucopolysaccharidosis (MPS) are rare inherited diseases characterized by accumulation of lysosomal glycosaminoglycans, including heparan sulfate (HS). Patients exhibit progressive multi-visceral dysfunction and shortened lifespan mainly due to a severe cardiac/respiratory decline. Cathepsin V (CatV) is a potent elastolytic protease implicated in extracellular matrix (ECM) remodeling. Whether CatV is inactivated by HS in lungs from MPS patients remained unknown. Herein, CatV colocalized with HS in MPS bronchial epithelial cells. HS level correlated positively with the severity of respiratory symptoms and negatively to the overall endopeptidase activity of cysteine cathepsins. HS bound tightly to CatV and impaired its activity. Withdrawal of HS by glycosidases preserved exogenous CatV activity, while addition of Surfen, a HS antagonist, restored elastolytic CatV-like activity in MPS samples. Our data suggest that the pathophysiological accumulation of HS may be deleterious for CatV-mediated ECM remodeling and for lung tissue homeostasis, thus contributing to respiratory disorders associated to MPS diseases.
dc.relation.ispartofThe Linhardt Research Labs Online Collection
dc.relation.ispartofRensselaer Polytechnic Institute, Troy, NY
dc.subjectChemistry and chemical biology
dc.subjectChemical and biological engineering
dc.subjectBiomedical engineering
dc.titleThe abnormal accumulation 1 of heparan sulfate in patients with mucopolysaccharidosis prevents the elastolytic activity of cathepsin V
dc.rights.holderIn Copyright : this Item is protected by copyright and/or related rights. You are free to use this Item in any way that is permitted by the copyright and related rights legislation that applies to your use. For other uses you need to obtain permission from the rights-holder(s).
dc.relation.departmentThe Linhardt Research Labs.
dc.relation.departmentThe Shirley Ann Jackson, Ph.D. Center for Biotechnology and Interdisciplinary Studies (CBIS)

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