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Effects of Serum from a Fibrodysplasia Ossificans Progressiva Patient on Osteoblastic Cells

Open Journal of Endocrine and Metabolic Diseases 2012年第1期2卷 1-6页

作者： Itoko Hisa Akira Kawara takenobu katagiri Toshitsugu Sugimoto Hiroshi Kaji Division of Diabetes and Endocrinology Department of Internal Medicine Kobe University Graduate School of Medicine Kobe Japan Division of Pathophysiology Research Center for Genomic Medicine Saitama Medical University Japan Internal Medicine 1 Shimane University Faculty of Medicine Izumo Japan Kawara Clinic Kobe Japan

Introduction: Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Patients and Methods: In the present study, we present a case of FOP with marked progressive ossification of extraskeletal tissues. We investigated whether soluble factors in serum would affect the osteoblast phenotype by examining the effects of serum from this patient and control subjects on mouse osteoblastic MC3T3-E1 cells. Results: The clinical findings of this patient were compatible with FOP, and direct sequence analysis of genomic DNA demonstrated the presence of a heterozygous 617G> A (R206H) mutation of activin type 1 receptor (ACVR1). Serum from the FOP patient enhanced the level of alkaline phosphatase (ALP) in Western blotting, compared with serum from the control, in MC3T3-E1 cells. Moreover, serum from the FOP patient enhanced the levels of ALP, osteocalcin and bone morphogenetic protein-2 mRNA in these cells. Conclusion: We presented a case of FOP with progressive ossification in extra-skeletal tissues with ACVR1 mutation. The present data suggest that the serum from this patient includes some soluble factors, which might enhance the osteoblast differentiation and BMP-2 expression in mouse osteoblastic cells.

关键词： Fibrodysplasia Ossificans Progressiva Osteoblast Bone Morphogenetic Protein

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Fungal pyrrolidine-containing metabolites inhibit alkaline phosphatase activity in bone morphogenetic protein-stimulated myoblastoma cells

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Acta Pharmaceutica Sinica B 2012年第1期2卷 23-27页

作者： Takashi Fukuda Ryuji Uchida Hiroyo Inoue Satoshi Ohte Hiroyuki Yamazaki Daisuke Matsuda takenobu katagiri Hiroshi Tomoda Graduate School of Pharmaceutical Sciences Kitasato UniversityTokyoJapan Research Center for Genomic Medicine Saitama Medical UniversitySaitamaJapan

Fibrodysplasia ossificans progressiva(FOP)is a rare autosomal dominant congenital disorder characterized by progressive heterotopic ossification in muscle tissues.A constitutively activated mutation of a bone morphogenetic protein(BMP)receptor,ALK2,has been identified in patients with *** report here that four structurally related compounds,lucilactaene,hydroxylucilactaene,NG-391 and NG-393,produced by fungal strain Fusarium sp.B88,inhibit BMP signaling in *** phosphatase activity,a marker enzyme of osteoblastic differentiation,was decreased in C2C12 myoblasts stably expressing mutant ALK2 by treatment with those compounds with IC_(50) values of 5.7,6.8,6.9 and 6.1 mM,***,NG-391 and NG-393 inhibited BMP-specific luciferase reporter activity,which is directly regulated by transcription factor Smads,with IC50 values of 1.4 and 2.1 mM,*** findings suggest that these fungal metabolites may provide a new direction in the development of FOP therapeutics.

关键词： Lucilactaenes Fungal metabolites C2C12 myoblasts Fibrodysplasia ossificans progressiva

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