[Fibrodysplasia ossificans progressiva and osteoimmunology].
Fibrodysplasia ossificans progressive (FOP) is a genetic disorder characterized by progressive heterotopic ossification (HO) in skeletal muscle, tendons and ligaments. FOP is caused by gain-of-function mutations of ALK2, a receptor of bone morphogenetic proteins. Immune responses have been suggested to be involved in HO in FOP, because muscle trauma induces acute HO in patients with FOP. Recently, Activin A, a non-osteogenic ligand, was identified as a ligand of the mutated ALK2 to induce HO. It was suggested that Activin A is a novel interface between FOP and osteoimmunology.
Division of Pathophysiology, Research Center for Genomic Medicine, Saitama Medical University, Japan.
Takenobu
T
Katagiri
D028341:Activins / Q000235:genetics*; D000818:Animals; D019485:Bone Morphogenetic Proteins / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D009154:Mutation / Q000235:genetics*; D009221:Myositis Ossificans / Q000235:genetics*; D009999:Ossification, Heterotopic / Q000235:genetics*
D019485:Bone Morphogenetic Proteins; C422405:activin A; D028341:Activins