In recessive OI types involving RIP, there is a distruption of the process by which transcription factors such as OASIS are translocated to the Golgi membrane upon ER stress, cleaved by S1P/S2P, and transported to the nucleus for transcription of genes for ER-stress response or UPR pathways [169]. Here, MBTPS2 is linked to osteogenesis imperfecta.