In contrast, in our study, along with the increase in BiP expression, activation of both transcription factors ATF4 (effector of PERK pathway) and ATF6 in cells with BiP upregulation was found, while expression of spliced forms of XBP1 (effector of IRE1α pathway) was predominant in OI type III as confirmed by real-time PCR and polyacrylamide gel electrophoresis of (reverse transcriptase) RT-PCR product. The gene discussed is ERN1; the disease is osteogenesis imperfecta type 3.