Altered Macrophage Function Associated with Crystalline Lung Inflammation in Acid Sphingomyelinase Deficiency

Deficiency of ASM (acid sphingomyelinase) causes the lysosomal storage Niemann-Pick disease (NPD). Patients with NPD type B may develop progressive interstitial lung disease with frequent respiratory infections. Although several investigations using the ASM-deficient (ASMKO) mouse NPD model revealed...

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Bibliografske podrobnosti
izdano v:Am J Respir Cell Mol Biol
Main Authors: Poczobutt, Joanna M., Mikosz, Andrew M., Poirier, Christophe, Beatman, Erica L., Serban, Karina A., Gally, Fabienne, Cao, Danting, McCubbrey, Alexandra L., Cornell, Christina F., Schweitzer, Kelly S., Berdyshev, Evgeny V., Bronova, Irina A., Paris, François, Petrache, Irina
Format: Artigo
Jezik:Inglês
Izdano: American Thoracic Society 2021
Teme:
Original Research
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC8086042/
https://ncbi.nlm.nih.gov/pubmed/33662226
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1165/rcmb.2020-0229OC
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