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000309977 1001_ $$00000-0001-9582-4448$$aWeiden, Eva-Maria$$b0
000309977 245__ $$aTRPML1 suppresses pulmonary fibrosis by limiting collagen and elastin deposition.
000309977 260__ $$a[London]$$bNature Publishing Group UK$$c2026
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000309977 520__ $$aIn pulmonary fibrosis lung tissue is thickened and scarred, and the lungs become progressively stiffer and smaller, leading to low levels of blood oxygen and shortness of breath. Lung fibrosis is not curable and life expectancy is reduced. Fibrosis is characterized by an increased accumulation of extracellular matrix (ECM) proteins such as collagen and elastin. ECM proteins are degraded predominantly by matrix metalloproteinases (MMPs). Here, we show that the lysosomal cation channel TRPML1, which causes the lysosomal storage disorder mucolipidosis type IV (MLIV) when mutated or lost, regulates the levels of MMPs in the ECM of mouse airways, modulating exocytosis of MMP2, 8, 9, 12, and 19, which mediate collagen/elastin degradation. While TRPML1 loss reduces MMP levels in lung macrophage and fibroblast supernatants, small molecule activation of TRPML1 results in increased levels. MLIV mice display a fibrosis-like lung phenotype similar to the phenotype evoked by bleomycin. We thus identify TRPML1 as a regulator of MMP release in the lung with loss of TRPML1 resulting in lung fibrosis due to excessive extracellular collagen and elastin accumulation.
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000309977 650_7 $$2Other$$aMcoln1
000309977 650_7 $$2Other$$aPulmonary Fibrosis
000309977 650_7 $$2Other$$aTRPML
000309977 650_7 $$2Other$$aTRPML1
000309977 650_7 $$2Other$$aTRPML3
000309977 7001_ $$00009-0009-5753-3637$$aSerianz, Zala$$b1
000309977 7001_ $$00000-0003-2779-3357$$aKlingl, Yvonne$$b2
000309977 7001_ $$aJörs, Simone$$b3
000309977 7001_ $$00000-0002-0685-7633$$aJaślan, Dawid$$b4
000309977 7001_ $$00000-0003-4792-3980$$aKeller, Marco$$b5
000309977 7001_ $$aCastro, Sandra Prat$$b6
000309977 7001_ $$00009-0008-7426-3088$$aMkhitaryan, Mane$$b7
000309977 7001_ $$aJeridi, Aicha$$b8
000309977 7001_ $$00000-0002-2073-2335$$aBriukhovetska, Daria$$b9
000309977 7001_ $$aSpix, Barbara$$b10
000309977 7001_ $$aScotto Rosato, Anna$$b11
000309977 7001_ $$aAgami, Ahmed$$b12
000309977 7001_ $$aSchiller, Herbert B$$b13
000309977 7001_ $$aRajan, Suhasini$$b14
000309977 7001_ $$00000-0002-6657-0466$$aSchredelseker, Johann$$b15
000309977 7001_ $$00000-0001-5414-1149$$aFois, Giorgio$$b16
000309977 7001_ $$aFrick, Manfred$$b17
000309977 7001_ $$0P:(DE-HGF)0$$aKobold, Sebastian$$b18
000309977 7001_ $$00000-0002-3919-8427$$aKlein, Margarethe$$b19
000309977 7001_ $$aGeisler, Fabian$$b20
000309977 7001_ $$00009-0006-9232-2198$$aGarcia-Fortanet, Jorge$$b21
000309977 7001_ $$aMurphy, Leon O$$b22
000309977 7001_ $$00000-0003-0009-8629$$aBracher, Franz$$b23
000309977 7001_ $$aWahl-Schott, Christian$$b24
000309977 7001_ $$00000-0002-0323-7965$$aGudermann, Thomas$$b25
000309977 7001_ $$00000-0002-1168-8707$$aDietrich, Alexander$$b26
000309977 7001_ $$00000-0002-9974-3052$$aBiel, Martin$$b27
000309977 7001_ $$00000-0003-1969-480X$$aYildirim, Ali Önder$$b28
000309977 7001_ $$00000-0002-0177-5559$$aGrimm, Christian$$b29
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