title: Hereditary gingival fibromatosis: characteristics and novel putative pathogenic mechanisms.
abstract: Hereditary gingival fibromatosis (HGF) is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and esthetic concerns. Histological and cell culture studies have uncovered some of the molecular and cellular changes associated with HGF. However, the pathogenesis of the disease is still largely unknown. Recent studies about the genetic characteristics of HGF have provided novel clues about the potential pathogenic mechanisms. In particular, mutation in the son-of-sevenless (SOS-1) gene has been associated with one form of the disease. However, HGF displays genetic heterogeneity, and mutations in other genes are also likely involved. This review outlines the current knowledge about the histological, cellular, and genetic characteristics of HGF. In addition, the potential role of the SOS-1 molecule and related novel intracellular signaling pathways in the pathogenesis of HGF will be discussed.
authors.affiliation: University of British Columbia, Faculty of Dentistry, Department of Oral Biological and Medical Sciences, Laboratory of Periodontal Biology, Vancouver, BC, Canada V6T 1Z3. lhakkine@interchange.ubc.ca
authors.forename: L
authors.initials: L
authors.lastname: Häkkinen
authors.forename: A
authors.initials: A
authors.lastname: Csiszar
mesh_terms: D049109:Cell Proliferation; D005347:Fibroblasts / Q000473:pathology; D005351:Fibromatosis, Gingival / Q000235:genetics* / Q000473:pathology; D018740:Genetic Heterogeneity; D006801:Humans; D020928:Mitogen-Activated Protein Kinases / Q000235:genetics; D009154:Mutation / Q000235:genetics; D018125:Receptors, Transforming Growth Factor beta / Q000235:genetics; D020837:SOS1 Protein / Q000235:genetics; D016212:Transforming Growth Factor beta / Q000235:genetics
chemical_list: D018125:Receptors, Transforming Growth Factor beta; D020837:SOS1 Protein; D016212:Transforming Growth Factor beta; D020928:Mitogen-Activated Protein Kinases