Hermansky-Pudlak syndrome type 1 causes impaired anti-microbial immunity and inflammation due to dysregulated immunometabolism

Hermansky-Pudlak syndrome type 1 causes impaired anti-microbial immunity and inflammation due to dysregulated immunometabolism

Hermansky-Pudlak syndrome (HPS) types 1 and 4 are caused by defective vesicle trafficking. The mechanism for Crohn's disease-like inflammation, lung fibrosis, and macrophage lipid accumulation in these patients remains enigmatic. The aim of this study is to understand the cellular basis of infl...

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Bibliographic Details
Main Authors: Cavounidis, A, Pandey, S, Capitani, M, Friedrich, M, Cross, A, Gartner, L, Aschenbrenner, D, Kim-Schulze, S, Lam, YK, Berridge, G, McGovern, DPB, Kessler, B, Fischer, R, Klenerman, P, Hester, J, Issa, F, Torres, EA, Powrie, F, Gochuico, BR, Gahl, WA, Cohen, L, Uhlig, HH
Format: Journal article
Language:English
Published: Elsevier 2022

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