دورية أكاديمية
Progeria and accelerated cardiovascular aging
العنوان: | Progeria and accelerated cardiovascular aging |
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المؤلفون: | Brandon K Walther, Yanhui Li, Rajarajan Amirthalingam Thandavarayan, John P Cooke |
المصدر: | Cardiology Plus, Vol 3, Iss 3, Pp 81-89 (2018) |
بيانات النشر: | Wolters Kluwer Health/LWW, 2018. |
سنة النشر: | 2018 |
المجموعة: | LCC:Diseases of the circulatory (Cardiovascular) system |
مصطلحات موضوعية: | Atherosclerosis, cardiovascular aging, cardiovascular disease, nuclear lamina, progeria, progerin, telomere, Diseases of the circulatory (Cardiovascular) system, RC666-701 |
الوصف: | Hutchinson–Gilford Progeria syndrome (HGPS) is characterized by accelerated aging leading to death in the teen years, usually due to severe coronary and/or carotid disease. The clinical presentation includes stunted growth, alopecia, loss of subcutaneous fat, osteoporosis, and cardiovascular disorders occurring in late childhood and the teen years including hypertension and accelerated vascular aging that precipitates myocardial infarction and cerebrovascular attacks. The disease is a nuclear laminopathy, due to a Lmna gene mutation. The aberrant protein (Progerin) accumulates in and distorts the nuclear envelope. We review the genetic and biochemical mechanism of HGPS; the clinical presentation with special attention to the cardiovascular pathology and complications; current therapeutic developments to address the disease; and the results of the clinical trials attempting to translate basic research insights into therapeutic benefit. An understanding of HGPS may lead to better treatment of other age-related disorders, particularly cardiovascular diseases. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2470-7511 2470-752X |
العلاقة: | http://www.cardiologyplus.org/article.asp?issn=2470-7511;year=2018;volume=3;issue=3;spage=81;epage=89;aulast=WaltherTest; https://doaj.org/toc/2470-7511Test; https://doaj.org/toc/2470-752XTest |
DOI: | 10.4103/cp.cp_26_18 |
الوصول الحر: | https://doaj.org/article/a11d28ec087b49cb9cc181ebfd4df03aTest |
رقم الانضمام: | edsdoj.11d28ec087b49cb9cc181ebfd4df03a |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 24707511 2470752X |
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DOI: | 10.4103/cp.cp_26_18 |