کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1929208 1050448 2012 4 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Detection of the dystroglycanopathy protein, fukutin, using a new panel of site-specific monoclonal antibodies
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
Detection of the dystroglycanopathy protein, fukutin, using a new panel of site-specific monoclonal antibodies
چکیده انگلیسی

Mutations in the gene encoding fukutin protein cause Fukuyama muscular dystrophy, a severe congenital disorder that occurs mainly in Japan. A major consequence of the mutation is reduced glycosylation of alpha-dystroglycan, which is also a feature of other forms of congenital and limb-girdle muscular dystrophy. Immunodetection of endogenous fukutin in cells and tissues has been difficult and this has hampered progress in understanding fukutin function and disease pathogenesis. Using a new panel of monoclonal antibodies which bind to different defined sites on the fukutin molecule, we now show that fukutin has the predicted size for a protein without extensive glycosylation and is present at the Golgi apparatus at very low levels. These antibodies should enable more rapid future progress in understanding the molecular function of fukutin.


► We have produced a new panel of twelve monoclonal antibodies against human fukutin.
► We have mapped their binding sites on fukutin using phage-displayed peptides.
► Endogenous fukutin is present in cells and tissues at very low levels.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 424, Issue 2, 27 July 2012, Pages 354–357
نویسندگان
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