کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5506429 1400294 2017 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Prion-like disorders and Transmissible Spongiform Encephalopathies: An overview of the mechanistic features that are shared by the various disease-related misfolded proteins
ترجمه فارسی عنوان
اختلالات پریون مانند و انسفالوپاتی های اسپوندوئید قابل انتقال: یک مرور کلی از ویژگی های مکانیکی که توسط پروتئین های گوناگون مرتبط با بیماری های مختلف به اشتراک گذاشته می شوند
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
چکیده انگلیسی
Prion diseases or Transmissible Spongiform Encephalopathies (TSEs) are a group of fatal neurodegenerative disorders affecting several mammalian species. Its causative agent, disease-associated prion protein (PrPd), is a self-propagating β-sheet rich aberrant conformation of the cellular prion protein (PrPC) with neurotoxic and aggregation-prone properties, capable of inducing misfolding of PrPC molecules. PrPd is the major constituent of prions and, most importantly, is the first known example of a protein with infectious attributes. It has been suggested that similar molecular mechanisms could be shared by other proteins implicated in diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis or systemic amyloidoses. Accordingly, several terms have been proposed to collectively group all these disorders. Through the stringent evaluation of those aspects that characterise TSE-causing prions, in particular propagation and spread, strain variability or transmissibility, we will discuss whether terms such as “prion”, “prion-like”, “prionoid” or “propagon” can be used when referring to the aetiological agents of the above other disorders. Moreover, it will also be discussed whether the term “infectious”, which defines a prion essential trait, is currently misused when referring to the other misfolded proteins.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 483, Issue 4, 19 February 2017, Pages 1125-1136
نویسندگان
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