کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2204829 1100827 2011 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Fusing a lasting relationship between ER tubules
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی بیولوژی سلول
پیش نمایش صفحه اول مقاله
Fusing a lasting relationship between ER tubules
چکیده انگلیسی

Atlastin is an integral membrane GTPase localized to the endoplasmic reticulum (ER). In vitro and in vivo analyses indicate that atlastin is a membrane fusogen capable of driving membrane fusion, suggesting a role in ER structure and maintenance. Interestingly, mutations in the human atlastin-1 gene, SPG3A, cause a form of autosomal dominant hereditary spastic paraplegia (HSP). The etiology of HSP is unclear, but two predominant forms of the disorder are caused by mutant proteins that affect ER structure, formation and maintenance in motor neurons. In this review, we describe the current knowledge about the molecular mechanism of atlastin function and its potential role in HSP. Greater understanding of the function of atlastin and associated proteins should provide important insight into normal ER biogenesis and maintenance, as well as the pathology of disease.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: - Volume 21, Issue 7, July 2011, Pages 416–423
نویسندگان
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