کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
8259577 1534641 2015 35 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
The neuronal ceroid lipofuscinoses: Opportunities from model systems
ترجمه فارسی عنوان
لیپوفوس کینوزهای سرود نورونی: فرصت های از سیستم های مدل
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
چکیده انگلیسی
The neuronal ceroid lipofuscinoses are a group of severe and progressive neurodegenerative disorders, generally with childhood onset. Despite the fact that these diseases remain fatal, significant breakthroughs have been made in our understanding of the genetics that underpin these conditions. This understanding has allowed the development of a broad range of models to study disease processes, and to develop new therapeutic approaches. Such models have contributed significantly to our knowledge of these conditions. In this review we will focus on the advantages of each individual model, describe some of the contributions the models have made to our understanding of the broader disease biology and highlight new techniques and approaches relevant to the study and potential treatment of the neuronal ceroid lipofuscinoses. This article is part of a Special Issue entitled: “Current Research on the Neuronal Ceroid Lipofuscinoses (Batten Disease)”.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1852, Issue 10, Part B, October 2015, Pages 2267-2278
نویسندگان
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