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Cartilage-hair hypoplasia is associated with severe dwarfism caused by impaired skeletal development. However, it is not clear why mutations in<jats:italic>RMRP<\/jats:italic>RNA lead to skeletal dysplasia. Since chondrogenic differentiation of the growth plate is required for development of long bones, we hypothesized that<jats:italic>RMRP<\/jats:italic>RNA plays a pivotal role in chondrogenic differentiation. Expression of<jats:italic>Rmrp<\/jats:italic>RNA and RNase MRP protein subunits was detected in the murine growth plate and during the course of chondrogenic differentiation of ATDC5 cultures, where<jats:italic>Rmrp<\/jats:italic>RNA expression was found to be correlated with chondrocyte hypertrophy. Genetic interference with<jats:italic>Rmrp<\/jats:italic>RNA expression in ATDC5 cultures caused a deregulation of chondrogenic differentiation, with a prominent impact on hypertrophy and changes in pre-rRNA processing and rRNA levels. Promoter reporter studies showed that<jats:italic>Rmrp<\/jats:italic>RNA expression responds to chondrogenic morphogens. Chondrogenic trans-differentiation of cartilage-hair hypoplasia fibroblasts was impaired with a pronounced impact on hypertrophic differentiation. Together, our data show that<jats:italic>RMRP<\/jats:italic>RNA expression is regulated during different stages of chondrogenic differentiation and indicate that<jats:italic>RMRP<\/jats:italic>RNA may play a pivotal role in chondrocyte hypertrophy, with potential consequences for CHH pathobiology.<\/jats:p>","DOI":"10.1038\/s41598-017-06809-5","type":"journal-article","created":{"date-parts":[[2017,7,19]],"date-time":"2017-07-19T14:09:04Z","timestamp":1500473344000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":56,"title":["Expression of RMRP RNA is regulated in chondrocyte hypertrophy and determines chondrogenic differentiation"],"prefix":"10.1038","volume":"7","author":[{"given":"Mandy M. F.","family":"Steinbusch","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjolein M. 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