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

ABSTRACT: 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 inflammation and foamy macrophages, there is little insight into the pathogenesis of NPD-associated lung disease. Using ASMKO mice, we report that ASM deficiency is associated with a complex inflammatory phenotype characterized by marked accumulation of monocyte-derived CD11b⁺ macrophages and expansion of airspace/alveolar CD11c⁺ CD11b^- macrophages, both with increased size, granularity, and foaminess. Both the alternative and classical pathways were activated,

SUBMITTER: Poczobutt JM 

PROVIDER: S-EPMC8086042 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature