Given the prior demonstration that transcription factors related to lipid, such as SREBPs, are also not well-cleaved in type XVIII OI-affected osteoblasts [171], this may speak to a tissue-specific critical function distal to RIP cleavage of OASIS that makes a specific set of S2P mutations crucial for bone formation. The gene discussed is MBTPS2; the disease is osteogenesis imperfecta.