کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
10956890 1099462 2005 9 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Disrupted compaction of CNS myelin in an OSP/Claudin-11 and PLP/DM20 double knockout mouse
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی بیولوژی سلول
پیش نمایش صفحه اول مقاله
Disrupted compaction of CNS myelin in an OSP/Claudin-11 and PLP/DM20 double knockout mouse
چکیده انگلیسی
OSP/claudin-11 and PLP are both tetraspan proteins concentrated in CNS myelin. It has been proposed that they have a structural role in myelin formation and maintenance due to their localization and concentration in membrane sheaths. This hypothesis is not supported by the fact that both OSP/claudin-11- and PLP-null mice have relatively normal-appearing myelin and mild neurological deficits. Since both OSP/claudin-11 and PLP are abundant in myelin and have similar structures, the mild phenotypes of the knockout mice are likely due to compensatory mechanisms. Here we show that when both OSP/claudin-11 and PLP genes are knocked out, mice had severe neurological deficits, markedly abnormal myelin compaction, and smaller axon diameters. Interestingly, when either of these genes was knocked out, the expression of the other protein was increased. These data demonstrate that OSP/claudin-11 and PLP have essential structural functions in maintaining normal compact myelin and there is redundancy in their functions.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular and Cellular Neuroscience - Volume 29, Issue 3, July 2005, Pages 405-413
نویسندگان
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