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Recent Topics in Fibrodysplasia Ossificans Progressiva
Takenobu Katagiri,Sho Tsukamoto,Yutaka Nakachi,Mai Kuratani 대한내분비학회 2018 Endocrinology and metabolism Vol.33 No.3
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease that is characterized by the formation of heterotopic bone tissuesin soft tissues, such as skeletal muscle, ligament, and tendon. It is difficult to remove such heterotopic bones via internal medicineor invasive procedures. The identification of activin A receptor, type I (ACVR1)/ALK2 gene mutations associated with FOP hasallowed the genetic diagnosis of FOP. The ACVR1/ALK2 gene encodes the ALK2 protein, which is a transmembrane kinase receptorin the transforming growth factor-β family. The relevant mutations activate intracellular signaling in vitro and induce heterotopicbone formation in vivo. Activin A is a potential ligand that activates mutant ALK2 but not wild-type ALK2. Various types of smallchemical and biological inhibitors of ALK2 signaling have been developed to establish treatments for FOP. Some of these are inclinical trials in patients with FOP.
Yamada, Atsushi,Takami, Masamichi,Kawawa, Tadaharu,Yasuhara, Rika,Zhao, Baohong,Mochizuki, Ayako,Miyamoto, Yoichi,Eto, Tomoo,Yasuda, Hisataka,Nakamichi, Yuko,Kim, Nacksung,Katagiri, Takenobu,Suda, Tat Blackwell Publishing Ltd 2007 Immunology Vol.120 No.4
<P>Summary</P><P>Interleukin (IL)-4 and IL-13 are closely related cytokines known to inhibit osteoclast formation by targeting osteoblasts to produce an inhibitor, osteoprotegerin (OPG), as well as by directly targeting osteoclast precursors. However, whether their inhibitory actions are the same remains unclear. The inhibitory effect of IL-4 was stronger than that of IL-13 in an osteoclast-differentiation culture system containing mouse osteoblasts and osteoclast precursors. Both cytokines induced OPG production by osteoblasts in similar time- and dose-dependent manners. However, IL-4 was stronger in direct inhibition that targeted osteoclast precursors. Furthermore, IL-4 induced phosphorylation of signal transducer and activator of transcription-6 (STAT6) at lower concentrations than those of IL-13 in osteoclast precursors. IL-4 but not IL-13 strongly inhibited the expression of nuclear factor of activated T-cells, cytoplasmic 1 (nuclear factor-ATc1), a key factor of osteoclast differentiation, by those precursors. Thus, the activities of IL-4 and IL-13 toward osteoclast precursors were shown to be different in regards to inhibition of osteoclast differentiation, whereas those toward osteoblasts for inducing OPG expression were equivalent.</P>