Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia
Author:
Affiliation:
1. Institut de Génétique et de Biologie Moléculaire et Cellulaire, France;
2. Université Libre de Bruxelles, Brussels, Belgium;
3. The Hospital for Sick Children, Toronto, ON, Canada
Abstract
Publisher
The Company of Biologists
Subject
General Biochemistry, Genetics and Molecular Biology,Immunology and Microbiology (miscellaneous),Medicine (miscellaneous),Neuroscience (miscellaneous)
Link
http://journals.biologists.com/dmm/article-pdf/6/3/608/1866207/608.pdf
Reference50 articles.
1. Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations;Abramov;Brain,2010
2. GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology;Al-Mahdawi;Genomics,2006
3. The first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxia;Calmels;PLoS ONE,2009
4. Friedreich’s ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion;Campuzano;Science,1996
5. Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes;Campuzano;Hum. Mol. Genet.,1997
Cited by 128 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献
1. Targeted genetic therapies for inherited disorders that affect both cardiac and skeletal muscle;Experimental Physiology;2023-12-14
2. Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain;Cell Death & Disease;2023-12-08
3. Patient-derived iPSC models of Friedreich ataxia: a new frontier for understanding disease mechanisms and therapeutic application;Translational Neurodegeneration;2023-09-20
4. How can we use stem cell-derived cardiomyocytes to understand the involvement of energetic metabolism in alterations of cardiac function?;Frontiers in Molecular Medicine;2023-09-01
5. Proprioceptors-enriched neuronal cultures from induced pluripotent stem cells from Friedreich ataxia patients show altered transcriptomic and proteomic profiles, abnormal neurite extension, and impaired electrophysiological properties;Brain Communications;2022-12-29
1.学者识别学者识别
2.学术分析学术分析
3.人才评估人才评估
"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370
www.globalauthorid.com
TOP
Copyright © 2019-2024 北京同舟云网络信息技术有限公司 京公网安备11010802033243号 京ICP备18003416号-3