کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4036024 | 1263566 | 2007 | 14 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Impaired photoreceptor protein transport and synaptic transmission in a mouse model of Bardet–Biedl syndrome
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
سیستم های حسی
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چکیده انگلیسی
Bardet–Biedl syndrome (BBS) is an oligogenic syndrome whose manifestations include retinal degeneration, renal abnormalities, obesity and polydactylia. Evidence suggests that the main etiopathophysiology of this syndrome is impaired intraflagellar transport (IFT). In this study, we study the Bbs4-null mouse and investigate photoreceptor structure and function after loss of this gene. We find that Bbs4-null mice have defects in the transport of phototransduction proteins from the inner segments to the outer segments, before signs of cell death. Additionally, we show defects in synaptic transmission from the photoreceptors to secondary neurons of the visual system, demonstrating multiple functions for BBS4 in photoreceptors.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Vision Research - Volume 47, Issue 27, December 2007, Pages 3394–3407
Journal: Vision Research - Volume 47, Issue 27, December 2007, Pages 3394–3407
نویسندگان
Muhammad M. Abd-El-Barr, Kristen Sykoudis, Sara Andrabi, Erica R. Eichers, Mark E. Pennesi, Perciliz L. Tan, John H. Wilson, Nicholas Katsanis, James R. Lupski, Samuel M. Wu,