The Role of GPR56 in Neurofibromatosis type 1 Secondary to Scoliosis

Background: Scoliosis is a common manifestation of neurofibromatosis type 1, causing significant morbidity. The etiology of dystrophic scoliosis in neurofibromatosis type 1(NF1) is not fully understood and its therapies are lacking. This article focused on how GPR56 affected the development of NF1 cells and the related factors of osteoblast and osteoclast. Methods: Through RNA-sequencing, G-protein-coupled receptor (GPR56) was found highly differential expressed in the NF1 with scoliosis samples. We measured the GPR56 how affected the NF1 cells in vitro. Then we tested the influence of GPR56 in osteoclasts and osteoblasts. Results: We reported that knockdown GPR56 promoted the proliferation and cell cycle progression of NF1 cell. Furthermore, knockdown GPR56 can inhibited the osteoblastic differentiation and osteoclast formation, but had greater influence on osteoblastic differentiation, which indicated that knockdown GPR56 can promote the deterioration of NF1 tumors, meanwhile break the dynamic balance of bone formation and bone absorption, leading to increased bone resorption and eventually scoliosis. Conclusions: In conclusion, we found that GPR56 may inhibit the proliferation and cell cycle progression of NF1. Furthermore, GPR56 can promote osteoblastic differentiation and osteoclast formation, but had greater influence on osteoblastic differentiation, which indicated that knockdown GPR56 can promote the deterioration of NF1 tumors, meanwhile break the dynamic balance of bone formation and bone absorption, leading to increased bone resorption.

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