Article
Response to Cysteamine in Osteoclasts Obtained from Patients with Nephropathic Cystinosis: A Genotype/Phenotype Correlation.
Cells - 21 Sept 2021
Quinaux Thomas, Bertholet-Thomas Aurélia, Servais Aude, Boyer Olivia, Vrillon Isabelle, Hogan Julien, Lemoine Sandrine, Gaillard Ségolène, Alioli Candide, Vasseur Sophie, Acquaviva Cécile, Peyruchaud Olivier, Machuca-Gayet Irma, Bacchetta Justine
Abstract excerpt
Bone complications of cystinosis have been recently described. The main objectives of this paper were to determine in vitro the impact of CTNS mutations and cysteamine therapy on human osteoclasts and to carry out a genotype-phenotype analysis related to osteoclastic differentiation. Human osteoclasts were differentiated from peripheral blood mononuclear cells (PBMCs) and were treated with increasing doses of...
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