sh3pxd2b mice are a model for craniofacial dysmorphology and otitis mediash3pxd2b小鼠颅面模型dysmorphology和中耳炎.pdfVIP

Sh3pxd2b Mice Are a Model for Craniofacial Dysmorphology and Otitis Media 1 2 1 2 2 2 2 Bin Yang , Cong Tian , Zhi-guang Zhang *, Feng-chan Han , Rami Azem , Heping Yu , Ye Zheng , Ge 3 2 2 Jin , James E. Arnold , Qing Y. Zheng * 1 Department of Oral and Maxillofacial Surgery, Guanghua School of Stomatology, Sun Yat-Sen University, Guangzhou, China, 2 Department of Otolaryngology - Head and Neck Surgery, School of Medicine, Case Western Reserve University, Cleveland, Ohio, United States of America, 3 School of Dental Medicine, Case Western Reserve University, Cleveland, Ohio, United States of America Abstract Craniofacial defects that occur through gene mutation during development increase vulnerability to eustachian tube dysfunction. These defects can lead to an increased incidence of otitis media. We examined the effects of a mutation in the Sh3pxd2b gene (Sh3pxd2bnee) on the progression of otitis media and hearing impairment at various developmental stages. We found that all mice that had the Sh3pxd2bnee mutation went on to develop craniofacial dysmorphologies and subsequently otitis media, by as early as 11 days of age. We found noteworthy changes in cilia and goblet cells of the middle ear mucosa in Sh3pxd2bnee mutant mice using scanning electronic microscopy. By measuring craniofacial dimensions, we determined for the first time in an animal model that this mouse has altered eustachian tube morphology consistent with a more horizontal position of the eustachian tube. All mutants were found to have hearing impairment. Expression of TNF-a and TLR2, which correlates with inflammati