Hereditary Gingival Fibromatosis

2021 ◽  
Vol 17 (1) ◽  
pp. 45-45
Keyword(s):  
Gingival Fibromatosis

GINGIVAL FIBROMATOSIS: AN EXTREME CASE

2016 ◽  
Author(s):  
Diogo Semedo
Keyword(s):  
Extreme Case ◽  
Gingival Fibromatosis

Gingival fibromatosis: a case report

2018 ◽  
Author(s):  
Omar Halouani
Keyword(s):  
Case Report ◽  
Gingival Fibromatosis

scholarly journals Activated KCNQ1 channel promotes fibrogenic response in hereditary gingival fibromatosis via clustering and activation of Ras

2020 ◽  
Author(s):  
Qian Gao ◽  
Chengcan Yang ◽  
Liuyan Meng ◽  
Ziming Wang ◽  
Dong Chen ◽  
...  
Keyword(s):  
Gingival Fibromatosis

Hereditary gingival fibromatosis

1964 ◽  
Vol 17 (3) ◽  
pp. 339-351 ◽  
Author(s):  
Peter F. Laband ◽  
George Habib ◽  
G.S. Humphreys
Keyword(s):  
Gingival Fibromatosis

Zimmermann-Laband Syndrome

2012 ◽  
Vol 36 (3) ◽  
pp. 297-300 ◽  
Author(s):  
K Sawaki ◽  
K Mishima ◽  
A Sato ◽  
Y Goda ◽  
A Osugi ◽  
...  
Keyword(s):  
Learning Difficulties ◽  
Joint Hypermobility ◽  
Rare Disorder ◽  
Early Presentation ◽  
Gingival Fibromatosis

Zimmermann–Laband syndrome is a very rare disorder characterized by gingival fibromatosis, abnormalities of soft cartilages of the nose and/or ears, hypoplastic or absent nails and terminal phalanges, joint hypermobility, hypatoslenomegaly, mild hirsutism and learning difficulties. Early presentation of Zimmer–mann–Laband syndrome in a newborn has rarely been described. This paper describes a newborn patient with Zimmermann–Laband syndrome.

Proliferative response to phenytoin and nifedipine in gingival fibroblasts cultured from humans with gingival fibromatosis

2004 ◽  
Vol 18 (4) ◽  
pp. 465-470 ◽  
Author(s):  
Masakazu Sano ◽  
Nozomi Ohuchi ◽  
Tomio Inoue ◽  
Kayoko Tono ◽  
Tetsuhiko Tachikawa ◽  
...  
Keyword(s):  
Proliferative Response ◽  
Gingival Fibroblasts ◽  
Gingival Fibromatosis

Symmetrical gingival fibromatosis

1981 ◽  
Vol 51 (1) ◽  
pp. 62-67 ◽  
Author(s):  
Alan R. Gould ◽  
Victor H. Escobar
Keyword(s):  
Gingival Fibromatosis

Novel REST Truncation Mutations Causing Hereditary Gingival Fibromatosis

2021 ◽  
pp. 002203452199662
Author(s):  
J.T. Chen ◽  
C.H. Lin ◽  
H.W. Huang ◽  
Y.P. Wang ◽  
P.C. Kao ◽  
...  
Keyword(s):  
Nuclear Localization ◽  
Genetic Disorder ◽  
Dominant Negative ◽  
Dominant Negative Effect ◽  
Wild Type ◽  
Negative Effect ◽  
Localization Signal ◽  
Gingival Fibromatosis

Hereditary gingival fibromatosis (HGF) is a rare genetic disorder featured by nonsyndromic pathological overgrowth of gingiva. The excessive gingival tissues can cause dental, masticatory, and phonetic problems, which impose severe functional and esthetic burdens on affected individuals. Due to its high recurrent rate, patients with HGF have to undergo repeated surgical procedures of gingival resection, from childhood to adulthood, which significantly compromises their quality of life. Unraveling the genetic etiology and molecular pathogenesis of HGF not only gains insight into gingival physiology and homeostasis but also opens avenues for developing potential therapeutic strategies for this disorder. Recently, mutations in REST (OMIM *600571), encoding a transcription repressor, were reported to cause HGF (GINGF5; OMIM #617626) in 3 Turkish families. However, the functions of REST in gingival homeostasis and pathogenesis of REST-associated HGF remain largely unknown. In this study, we characterized 2 HGF families and identified 2 novel REST mutations, c.2449C>T (p.Arg817*) and c.2771_2793dup (p.Glu932Lysfs*3). All 5 mutations reported to date are nonsenses or frameshifts in the last exon of REST and would presumably truncate the protein. In vitro reporter gene assays demonstrated a partial or complete loss of repressor activity for these truncated RESTs. When coexpressed with the full-length protein, the truncated RESTs impaired the repressive ability of wild-type REST, suggesting a dominant negative effect. Immunofluorescent studies showed nuclear localization of overexpressed wild-type and truncated RESTs in vitro, indicating preservation of the nuclear localization signal in shortened proteins. Immunohistochemistry demonstrated a comparable pattern of ubiquitous REST expression in both epithelium and lamina propria of normal and HGF gingival tissues despite a reduced reactivity in HGF gingiva. Results of this study confirm the pathogenicity of REST truncation mutations occurring in the last exon causing HGF and suggest the pathosis is caused by an antimorphic (dominant negative) disease mechanism.

Heterogeneity in Inherited Dental Traits, Gingival Fibromatosis and Amelogenesis Imperfecta

1971 ◽  
Vol 64 (Supplement) ◽  
pp. 16-25 ◽  
Author(s):  
CARL J. WITKOP
Keyword(s):  
Amelogenesis Imperfecta ◽  
Gingival Fibromatosis ◽  
Dental Traits
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