Article

Human iPSC-derived hypertrophic chondrocytes reveal a mutation-specific unfolded protein response in chondrodysplasias

2020-05-19

Abstract excerpt

<h4>Summary</h4> Chondrodysplasias are hereditary diseases caused by mutations in the components of growth cartilage. Although the unfolded protein response (UPR) has been identified as a key disease mechanism in mouse models, no suitable in vitro system has been reported to analyze the pathology in humans. Here, utilizing human chondrodysplasia-specific iPSCs, we examined the UPR caused by mutations in MATN3 o...

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Literature Corpus work: 26451b96-0472-5045-b714-8f1b1592f7de
DOI: 10.1101/2020.05.19.103960

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