Searchable abstracts of presentations at key conferences on calcified tissues

ba0004op5 | (1) | ICCBH2015

Osteoclast phenotype of giant multinucleated cells in cherubism may determine the disease aggressiveness

Kadlub Natacha , Sessiecq Quentin , Lehalle Daphne , Badoual Cecile , Majouffre Claire , Berdal Ariane , Vazquez Marie-Paule , Descroix Vianney , Picard Arnaud , Coudert Amelie

Cherubism is a rare genetic disorder characterized by extensive growth of a bilateral granuloma of the jaws, resulting in facial disfigurement.Histologically, the lesions consist of a fibrotic stroma with osteoclastic-like multinuclear giant cells (MGC). Cherubism is caused by gain-of-function mutations in the SH3BP2 protein. SH3BP2 is an intracellular adaptor protein positively regulating the activity of the nuclear factor of activated T-cells c1 (NFATc...