지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
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Paget’s disease of bone is characterized by highly localized areas of increasedbone resorption accompanied by exuberant, but aberrant new bone formationwith the primary cellular abnormality in osteoclasts. Paget’s disease provides animportant paradigm for understanding the molecular mechanisms regulatingboth osteoclast formation and osteoclast-induced osteoblast activity. Both genet-ic and environmental etiologies have been implicated in Paget’s disease, but theirrelative contributions are just beginning to be defined. To date, the only genewith mutations in the coding region linked to Paget’s disease is sequestosome-1(SQSTM1), which encodes the p62 protein, and these mutations lead to elevatedcytokine activation of NF-B in osteoclasts but do not induce a “pagetic osteoclast”phenotype. Further, genetic mutations linked to Paget’s appear insufficient tocause Paget’s disease and additional susceptibility loci or environmental factorsmay be required. Among the environmental factors suggested to induce Paget’sdisease, chronic measles (MV) infection has been the most studied. Expression ofthe measles virus nucleocapsid gene (MVNP) in osteoclasts induces pagetic-likeosteoclasts and bone lesions in mice. Further, mice expressing both MVNP in os-teoclasts and germline mutant p62 develop dramatic pagetic bone lesions thatwere strikingly similar to those seen in patients with Paget’s disease. Thus, interac-tions between environmental and genetic factors appear important to the devel-opment of Paget’s disease. In this article we review the mechanisms responsiblefor the effects of mutant p62 gene expression and MVNP on osteoclast and osteo-blast activity, and how they may contribute to the development of Paget’s diseaseof bone.
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참고문헌 (25)
참고문헌 신청
Albagha OM. / 2010 / Genome-wide association study identifies variants at CSF1, OPTN and TNFRSF11A as genetic risk factors for Paget's disease of bone / Nat Genet. 42 : 520
Albagha OM. / 2011 / Genome-wide association identifies three new susceptibility loci for Paget's disease of bone / Nat Genet. 43 : 685 ~ 689
Basle M. / 1978 / Structure and ultrastructure of osteoblasts and of the osteoid tissue in Paget's disease of bone (author's transl) / Pathol Biol (Paris). 26 : 475 ~ 479
Bolland MJ. / 2007 / Delayed development of Paget's disease in offspring inheriting SQSTM1 mutations / J Bone Miner Res. 22 : 411 ~ 415
Chung PY. / 2010 / Genetic variation in the TNFRSF11A gene encoding RANK is associated with susceptibility to Paget's disease of bone / J Bone Miner Res. 25 : 2592 ~ 2605
Albagha OM. / 2011 / Genome-wide association identifies three new susceptibility loci for Paget's disease of bone / Nat Genet. 43 : 685 ~ 689
Basle M. / 1978 / Structure and ultrastructure of osteoblasts and of the osteoid tissue in Paget's disease of bone (author's transl) / Pathol Biol (Paris). 26 : 475 ~ 479
Bolland MJ. / 2007 / Delayed development of Paget's disease in offspring inheriting SQSTM1 mutations / J Bone Miner Res. 22 : 411 ~ 415
Chung PY. / 2010 / Genetic variation in the TNFRSF11A gene encoding RANK is associated with susceptibility to Paget's disease of bone / J Bone Miner Res. 25 : 2592 ~ 2605
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