Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease
Ozen A, Kasap N, Vujkovic-Cvijin I, Apps R, Cheung F, Karakoc-Aydiner E, Akkelle B, Sari S, Tutar E, Ozcay F, Uygun DK, Islek A, Akgun G, Selcuk M, Sezer OB, Zhang Y, Kutluk G, Topal E, Sayar E, Celikel C, Houwen RHJ, Bingol A, Ogulur I, Eltan SB, Snow AL, Lake C, Fantoni G, Alba C, Sellers B, Chauvin SD, Dalgard CL, Harari O, Ni YG, Wang MD, Devalaraja-Narashimha K, Subramanian P, Ergelen R, Artan R, Guner SN, Dalgic B, Tsang J, Belkaid Y, Ertem D, Baris S, Lenardo MJ. Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease. Nature Immunology 2021, 22: 128-139. PMID: 33398182, PMCID: PMC7856263, DOI: 10.1038/s41590-020-00830-z.Peer-Reviewed Original ResearchConceptsBlockade of C5Complement C5 inhibitorInnate immune complement systemOveractivation of complementComplement regulatory proteins CD55Healthy gut microbiomeImmune complement systemCD55 deficiencyComplement hyperactivationImmune recoveryImmunoglobulin replacementGastrointestinal pathologyNormal immunityC5 inhibitionC5 inhibitorImmunoglobulin concentrationsMedical treatmentPathophysiological manifestationsInnate immunityLethal diseaseGut microbiomeProteins CD55Complement systemDiseaseHuman data
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