Cartilage Hair Hypoplasia (CHH) is an autosomal recessive disorder caused by mutations of the RNA component of the mitochondrial RNA processing endoribonuclease (RMRP) gene.284 RMRP is a long noncoding RNA (lncRNA) that participates in the formation of RNase-MRP complexes and tRNA maturation. Here, RMRP is linked to cartilage-hair hypoplasia.