کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10771166 | 1050839 | 2005 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
A new model mouse for Duchenne muscular dystrophy produced by 2.4Â Mb deletion of dystrophin gene using Cre-loxP recombination system
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
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![عکس صفحه اول مقاله: A new model mouse for Duchenne muscular dystrophy produced by 2.4Â Mb deletion of dystrophin gene using Cre-loxP recombination system A new model mouse for Duchenne muscular dystrophy produced by 2.4Â Mb deletion of dystrophin gene using Cre-loxP recombination system](/preview/png/10771166.png)
چکیده انگلیسی
Duchenne muscular dystrophy (DMD) is caused by mutation in the 2.4-Mb dystrophin (DMD) gene [1]. This gene encodes a number of tissue-specific isoforms of dystrophin generated by transcription from at least seven promoters and also by alternative splicing. We deleted entire genomic region of the DMD gene on mouse chromosome X using a Cre-loxP recombination system. Introduction of a loxP site in dystrophin's first and last exon by homologous recombination in mouse embryonic stem (ES) cells generated “DMD-floxed” (flanked by loxP sites) ES cells, which we subjected to Cre-mediated excision leading to establishment of “DMD-null” ES cell lines. The DMD-null mice produced from the DMD-null ES cells were viable but displayed severe muscular hypertrophy and dystrophy. In addition to the muscular impairment, the DMD-null mouse exhibited some behavioral abnormality and male sterility. The DMD-floxed mice produced from the DMD-floxed ES cells were viable, phenotypically normal, and were born with the expected Mendelian frequency, despite the absence of brain (cortical)-type dystrophin (Dp427c) expression. Since production of multiple dystrophin isoforms due to alternative splicing or exon skipping is totally prevented in the DMD-null mouse, these new mutants will provide an improved model system for functional studies of dystrophin and its isoforms.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 328, Issue 2, 11 March 2005, Pages 507-516
Journal: Biochemical and Biophysical Research Communications - Volume 328, Issue 2, 11 March 2005, Pages 507-516
نویسندگان
Hiroe Kudoh, Haruko Ikeda, Makoto Kakitani, Akiko Ueda, Michiko Hayasaka, Kazuma Tomizuka, Kazunori Hanaoka,