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 inflammat...

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Veröffentlicht in:	Mucosal immunology 2022-06, Vol.15 (6), p.1431-1446
Hauptverfasser:	Cavounidis, Athena, Pandey, Sumeet, Capitani, Melania, Friedrich, Matthias, Cross, Amy, Gartner, Lisa, Aschenbrenner, Dominik, Kim-Schulze, Seunghee, Lam, Ying Ka, Berridge, Georgina, McGovern, Dermot P. B., Kessler, Benedikt, Fischer, Roman, Klenerman, Paul, Hester, Joanna, Issa, Fadi, Torres, Esther A., Powrie, Fiona, Gochuico, Bernadette R., Gahl, William A., Cohen, Louis, Uhlig, Holm H.
Format:	Artikel
Sprache:	eng
Schlagworte:	Allergology Antibodies Bioenergetics Biomedical and Life Sciences Biomedicine Blood cells Cell lines Colitis Crohn's disease Cytometry Fibrosis Gastroenterology Granuloma Granulomas Hermanski-Pudlak Syndrome - complications Hermanski-Pudlak Syndrome - genetics Hermanski-Pudlak Syndrome - pathology Hermansky-Pudlak syndrome Humans Immunity Immunology Inflammation Inflammatory bowel diseases Lipids Lung diseases Lysosomes Macrophages Metabolism Monocytes Patients Peripheral blood Phenotypes Proteomics TOR protein TOR Serine-Threonine Kinases Transcriptomics Tumor necrosis factor
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