کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4327169 | 1614111 | 2010 | 14 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Ush1c gene expression levels in the ear and eye suggest different roles for Ush1c in neurosensory organs in a new Ush1c knockout mouse
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کلمات کلیدی
ERGABROHCauditory-evoked brainstem responseRetinitis pigmentosaelectroretinogram - الکتروترینوگرامIHC - ایمونوهیستوشیمیOuter hair cells - سلول های موی بیرونیinner hair cells - سلول های موی درونیSEM - مدل معادلات ساختاری / میکروسکوپ الکترونی روبشیScanning electron microscopy - میکروسکوپ الکترونی روبشی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله

چکیده انگلیسی
Usher syndrome (USH) is the most common form of deaf-blindness in humans. Molecular characterization revealed that the USH gene products form a macromolecular protein network in hair cells of the inner ear and in photoreceptor cells of the retina via binding to PDZ domains in the scaffold protein harmonin encoded by the Ush1c gene in mice and humans. Although several mouse mutants for the Ush1c gene have been described, we generated a targeted null mutation Ush1c mouse model in which the first four exons of the Ush1c gene were replaced with a reporter gene. Here, we assessed the expression pattern of the reporter gene under control of Ush1c regulatory elements and characterized the phenotype of mice defective for Ush1c. These Ush1 knockout mice are deaf but do not recapitulate vision defects before 10Â months of age. Our data show LacZ expression in multiple layers of the retina but in neither outer nor inner segments of the photoreceptor layers in mice bearing the knockout construct at 1-5Â months of age. The fact that Ush1c expression is much higher in the ear than in the eye suggests a different role for Ush1c in ear function than in the eye and may explain why Ush1c mutant mice do not recapitulate vision defects.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain Research - Volume 1328, 30 April 2010, Pages 57-70
Journal: Brain Research - Volume 1328, 30 April 2010, Pages 57-70
نویسندگان
Cong Tian, Xue Z. Liu, Fengchan Han, Heping Yu, Chantal Longo-Guess, Bin Yang, Changjun Lu, Denise Yan, Qing Y. Zheng,