A Rare Skeletal Disorder, Fibrous Dysplasia: A Review of Its Pathogenesis and Therapeutic Prospects

被引:12
作者
Kim, Ha-Young [1 ,2 ]
Shim, Jung-Hee [3 ,4 ]
Heo, Chan-Yeong [1 ,2 ,3 ]
机构
[1] Seoul Natl Univ, Interdisciplinary Program Bioengn, Seoul 08826, South Korea
[2] Seoul Natl Univ, Coll Med, Dept Plast & Reconstruct Surg, Seoul 03080, South Korea
[3] Seoul Natl Univ, Bundang Hosp, Dept Plast & Reconstruct Surg, Seongnam 13620, South Korea
[4] Seoul Natl Univ, Dept Res Adm Team, Bundang Hosp, Seongnam 13620, South Korea
关键词
fibrous dysplasia; bone disorder; GNAS mutation; variant G protein; rare disease; pathogenesis; NERVE GROWTH-FACTOR; MCCUNE-ALBRIGHT SYNDROME; BONE CANCER PAIN; GS-ALPHA; PAMIDRONATE TREATMENT; SMOOTH-MUSCLE; STEM-CELLS; ADIPOGENIC DIFFERENTIATION; OSTEOGENIC DIFFERENTIATION; INTRAVENOUS PAMIDRONATE;
D O I
10.3390/ijms242115591
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Fibrous dysplasia (FD) is a rare, non-hereditary skeletal disorder characterized by its chronic course of non-neoplastic fibrous tissue buildup in place of healthy bone. A myriad of factors have been associated with its onset and progression. Perturbation of cell-cell signaling networks and response outputs leading to disrupted building blocks, incoherent multi-level organization, and loss of rigid structural motifs in mineralized tissues are factors that have been identified to participate in FD induction. In more recent years, novel insights into the unique biology of FD are transforming our understandings of its pathology, natural discourse of the disease, and treatment prospects. Herein, we built upon existing knowledge with recent findings to review clinical, etiologic, and histological features of FD and discussed known and potential mechanisms underlying FD manifestations. Subsequently, we ended on a note of optimism by highlighting emerging therapeutic approaches aimed at either halting or ameliorating disease progression.
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页数:26
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