{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T23:04:09Z","timestamp":1792623849719,"version":"4.5.0"},"reference-count":74,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T00:00:00Z","timestamp":1642377600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["GR4315\/4-1"],"award-info":[{"award-number":["GR4315\/4-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["GRK2338 P08"],"award-info":[{"award-number":["GRK2338 P08"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["SFB\/TRR152 P04"],"award-info":[{"award-number":["SFB\/TRR152 P04"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["GR4315\/2-2"],"award-info":[{"award-number":["GR4315\/2-2"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Endolysosomal cation channels are emerging as key players of endolysosomal function such as endolysosomal trafficking, fusion\/fission, lysosomal pH regulation, autophagy, lysosomal exocytosis, and endocytosis. Diseases comprise lysosomal storage disorders (LSDs) and neurodegenerative diseases, metabolic diseases, pigmentation defects, cancer, immune disorders, autophagy related diseases, infectious diseases and many more. Involvement in lung diseases has not been a focus of attention so far but recent developments in the field suggest critical functions in lung physiology and pathophysiology. Thus, loss of TRPML3 was discovered to exacerbate emphysema formation and cigarette smoke induced COPD due to dysregulated matrix metalloproteinase 12 (MMP-12) levels in the extracellular matrix of the lung, a known risk factor for emphysema\/COPD. While direct lung function measurements with the exception of TRPML3 are missing for other endolysosomal cation channels or channels expressed in lysosome related organelles (LRO) in the lung, links between those channels and important roles in lung physiology have been established such as the role of P2X4 in surfactant release from alveolar epithelial Type II cells. Other channels with demonstrated functions and disease relevance in the lung such as TRPM2, TRPV2, or TRPA1 may mediate their effects due to plasma membrane expression but evidence accumulates that these channels might also be expressed in endolysosomes, suggesting additional and\/or dual roles of these channels in cell and intracellular membranes. We will discuss here the current knowledge on cation channels residing in endolysosomes or LROs with respect to their emerging roles in lung disease.<\/jats:p>","DOI":"10.3390\/cells11020304","type":"journal-article","created":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T08:20:42Z","timestamp":1642407642000},"page":"304","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Endolysosomal Cation Channels and Lung Disease"],"prefix":"10.3390","volume":"11","author":[{"given":"Barbara","family":"Spix","sequence":"first","affiliation":[{"name":"Walther Straub Institute of Pharmacology and Toxicology, Faculty of Medicine, Ludwig-Maximilians-University, 80336 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aicha","family":"Jeridi","sequence":"additional","affiliation":[{"name":"Pneumology Center, Institute of Lung Biology and Disease, Helmholtz Zentrum M\u00fcnchen, 85764 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8819-7965","authenticated-orcid":false,"given":"Meshal","family":"Ansari","sequence":"additional","affiliation":[{"name":"Pneumology Center, Institute of Lung Biology and Disease, Helmholtz Zentrum M\u00fcnchen, 85764 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali \u00d6nder","family":"Yildirim","sequence":"additional","affiliation":[{"name":"Pneumology Center, Institute of Lung Biology and Disease, Helmholtz Zentrum M\u00fcnchen, 85764 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herbert B.","family":"Schiller","sequence":"additional","affiliation":[{"name":"Pneumology Center, Institute of Lung Biology and Disease, Helmholtz Zentrum M\u00fcnchen, 85764 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0177-5559","authenticated-orcid":false,"given":"Christian","family":"Grimm","sequence":"additional","affiliation":[{"name":"Walther Straub Institute of Pharmacology and Toxicology, Faculty of Medicine, Ludwig-Maximilians-University, 80336 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,1,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1038\/s41467-021-27860-x","article-title":"Lung emphysema and impaired macrophage elastase clearance in mucolipin 3 deficient mice","volume":"13","author":"Spix","year":"2022","journal-title":"Nat. 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