骨 Paget 病：骨免疫学和破骨细胞病理学。
Paget's Disease of Bone: Osteoimmunology and Osteoclast Pathology.
机构信息
Division of Rheumatology and Immunology, Department of Medicine, Duke University, DUMC #3874, 200 Trent Dr, Durham, NC, 27710, USA.
Duke University, Durham, NC, USA.
出版信息
Curr Allergy Asthma Rep. 2021 Mar 25;21(4):23. doi: 10.1007/s11882-021-01001-2.
PURPOSE OF REVIEW
The purpose of this review is to recognize clinical features of Paget's disease of bone and to describe how the osteoclast, a myeloid-derived cell responsible for bone resorption, contributes to the disease.
RECENT FINDINGS
Recent studies have identified several variants in SQSTM1, OPTN, and other genes that may predispose individuals to Paget's disease of bone; studies of these genes and their protein products have elucidated new roles for these proteins in bone physiology. Understanding the pathologic mechanisms in the Pagetic osteoclast may lead to the identification of future treatment targets for other inflammatory and autoimmune diseases characterized by abnormal bone erosion and/or osteoclast activation.
目的综述
本文旨在认识骨 Paget 病的临床特征，并描述破骨细胞这一负责骨吸收的髓系来源细胞如何促成该疾病。
最新发现
近期研究已鉴定出 SQSTM1、OPTN 等多个基因的多种变异，这些变异可能使个体易患骨 Paget 病；对这些基因及其蛋白产物的研究阐明了这些蛋白在骨生理学中的新作用。理解 Pagetic 破骨细胞的病理机制可能有助于鉴定其他以异常骨侵蚀和/或破骨细胞激活为特征的炎症性和自身免疫性疾病的未来治疗靶点。
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