نتایج جستجو برای: dentinogenesis imperfecta

تعداد نتایج: 5063  

Journal: :International Journal of Medical & Dental Case Reports 2016

2017

Purpose The American Academy of Pediatric Dentistry (AAPD) recognizes that pediatric dentists are uniquely qualified to manage the oral health care needs of children with heritable dental developmental anomalies. These children have multiple, complex problems as their dental conditions affect both form and function and can have significant psychological impact. These conditions may present earl...

2010

Purpose The American Academy of Pediatric Dentistry (AAPD) recognizes that pediatric dentists are uniquely qualified to manage the oral health care needs of children with heritable dental developmental anomalies. These children have multiple, complex problems as their dental conditions affect both form and function and can have significant psychological impact. These conditions may present earl...

Journal: :Intractable & rare diseases research 2015
Xianlong Shi Yanqin Lu Yanzhou Wang Yu-Ang Zhang Yuanwei Teng Wanshui Han Zhenzhong Han Tianyou Li Mei Chen Junlong Liu Fengling Fang Conghui Dou Xiuzhi Ren Jinxiang Han

Osteogenesis imperfecta (OI) is an inheritable connective tissue disorder with a broad clinical heterozygosis, which can be complicated by other connective tissue disorders like Ehlers-Danlos syndrome (EDS). OI/EDS are rarely documented. Most OI/EDS mutations are located in the N-anchor region of type I procollagen and predominated by glycine substitution. We identified a c.3521C>T (p.A1174V) h...

2016
Megana K Prasad Véronique Geoffroy Serge Vicaire Bernard Jost Michael Dumas Stéphanie Le Gras Marzena Switala Barbara Gasse Virginie Laugel-Haushalter Marie Paschaki Bruno Leheup Dominique Droz Amelie Dalstein Adeline Loing Bruno Grollemund Michèle Muller-Bolla Séréna Lopez-Cazaux Maryline Minoux Sophie Jung Frédéric Obry Vincent Vogt Jean-Luc Davideau Tiphaine Davit-Beal Anne-Sophie Kaiser Ute Moog Béatrice Richard Jean-Jacques Morrier Jean-Pierre Duprez Sylvie Odent Isabelle Bailleul-Forestier Monique Marie Rousset Laure Merametdijan Annick Toutain Clara Joseph Fabienne Giuliano Jean-Christophe Dahlet Aymeric Courval Mustapha El Alloussi Samir Laouina Sylvie Soskin Nathalie Guffon Anne Dieux Bérénice Doray Stephanie Feierabend Emmanuelle Ginglinger Benjamin Fournier Muriel de la Dure Molla Yves Alembik Corinne Tardieu François Clauss Ariane Berdal Corinne Stoetzel Marie Cécile Manière Hélène Dollfus Agnès Bloch-Zupan

BACKGROUND Orodental diseases include several clinically and genetically heterogeneous disorders that can present in isolation or as part of a genetic syndrome. Due to the vast number of genes implicated in these disorders, establishing a molecular diagnosis can be challenging. We aimed to develop a targeted next-generation sequencing (NGS) assay to diagnose mutations and potentially identify n...

2015
Hsiang-Yu Lin Chih-Kuang Chuang Yi-Ning Su Ming-Ren Chen Hui-Chin Chiu Dau-Ming Niu Shuan-Pei Lin

BACKGROUND Osteogenesis imperfecta (OI) is a congenital disorder characterized by increased bone fragility and low bone mass. METHODS The presence of COL1A1 or COL1A2 mutation was investigated by direct sequencing in 72 patients with OI type I, III, or IV (27 males and 45 females; age range 0.2-62 years) from 37 unrelated families. The clinical features of these patients were also recorded. ...

Journal: :BMB reports 2009
Young-Mi Chae Sun-Hee Heo Jae-Young Kim Jae-Mok Lee Hyun-Mo Ryoo Je-Yoel Cho

Deletion of smpd3 induces osteogenesis and dentinogenesis imperfecta in mice. smpd3 is highly elevated in the parietal bones of developing mouse calvaria, but not in sutural mesenchymes. Here, we examine the mechanism of smpd3 regulation, which involves BMP2 stimulation of Runx2. smpd3 mRNA expression increased in response to BMP2 treatment and Runx2 transfection in C2C12 cells. The Runx2-respo...

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