Article
Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease.
Nature immunology - 1 Feb 2021
Ozen Ahmet, Kasap Nurhan, Vujkovic-Cvijin Ivan, Apps Richard, Cheung Foo, Karakoc-Aydiner Elif, Akkelle Bilge, Sari Sinan, Tutar Engin, Ozcay Figen, Uygun Dilara Kocacik, Islek Ali, Akgun Gamze, Selcuk Merve, Sezer Oya Balci, Zhang Yu, Kutluk Gunsel, Topal Erdem, Sayar Ersin, Celikel Cigdem, Houwen Roderick H J, Bingol Aysen, Ogulur Ismail, Eltan Sevgi Bilgic, Snow Andrew L, Lake Camille, Fantoni Giovanna, Alba Camille, Sellers Brian, Chauvin Samuel D, Dalgard Clifton L, Harari Olivier, Ni Yan G, Wang Ming-Dauh, Devalaraja-Narashimha Kishor, Subramanian Poorani, Ergelen Rabia, Artan Reha, Guner Sukru Nail, Dalgic Buket, Tsang John, Belkaid Yasmine, Ertem Deniz, Baris Safa, Lenardo Michael J
Abstract excerpt
Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
