Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndrome

Author:

Schramm Elizabeth C.1,Roumenina Lubka T.234,Rybkine Tania234,Chauvet Sophie234,Vieira-Martins Paula2,Hue Christophe234,Maga Tara5,Valoti Elisabetta6,Wilson Valerie7,Jokiranta Sakari8,Smith Richard J. H.5,Noris Marina6,Goodship Tim9,Atkinson John P.1,Fremeaux-Bacchi Veronique23410

Affiliation:

1. Division of Rheumatology, Department of Medicine, Washington University School of Medicine, Saint Louis, MO;

2. INSERM UMRS 1138, Cordeliers Research Center, Paris, France;

3. Université Paris Descartes Sorbonne Paris-Cité, Paris, France;

4. Université Pierre et Marie Curie, Paris, France;

5. Molecular Otolaryngology and Renal Research Laboratories, and Rare Renal Disease Clinic, Departments of Pediatrics and Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA;

6. Laboratory of Immunology and Genetics of Transplantation and Rare Diseases, Mario Negri Institute for Pharmacological Research, Ranica Bergamo, Italy;

7. Northern Molecular Genetics Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom;

8. Department of Bacteriology and Immunology, Medicum, and Research Programs Unit, Immunobiology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;

9. Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom; and

10. Assistance Publique–Hôpitaux de Paris, Service d’Immunologie Biologique, Hôpital Européen Georges Pompidou, Paris, France

Abstract

Key Points C3 mutations in aHUS commonly result in impaired complement regulation, C3 consumption, and a poor renal outcome. C3 mutations tend to cluster at the protein surface and facilitate mapping of putative binding sites for the regulatory proteins.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Cited by 109 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

同舟云学术

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