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Expression profiles and potential functions of long non-coding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients

ABSTRACT: Expression profiles and potential functions of long non-coding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients

PROVIDER: PRJNA644609 | ENA |

REPOSITORIES: ENA

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Expression profiles and potential functions of long non-coding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients

Project description:Autoimmune pulmonary alveolar proteinosis (APAP) is a rare lung disease which may be closely associated with autoantibody. However, there is little known about the function of long non-coding RNAs (lncRNAs) in APAP.To further investigate the role of lncRNAs in APAP, we screened the differential expression profiles of lncRNAs and mRNAs in peripheral blood samples between five APAP patients and five matched healthy controls. We analyzed the regulatory networks of lncRNA-mRNA and lncRNA-miRNA-mRNA via bioinformatics methods. This study identified differentially expressed (DE) lncRNAs and their potential corresponding mRNAs to provide a basis for the study of APAP pathogenesis.

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A Genome-Wide CRISPR Screen Identifies DTX4 Modulating Alveolar Macrophage Cholesterol Efflux in Pulmonary Alveolar Proteinosis [CRISPR]

Project description:Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome characterized by impaired surfactant clearance, driven by dysfunctional cholesterol efflux in alveolar macrophages (AMs). However, the molecular determinants governing AM cholesterol homeostasis remain largely elusive. Here, through a genome-wide CRISPR activation screen in foamy macrophages and bulk RNA sequencing of AMs from PAP patients, we identify Deltex E3 Ubiquitin Ligase 4 (DTX4) as a pivotal regulator of cholesterol efflux in AMs. Adeno-associated virus (AAV) -mediated silencing of DTX4 led to excessive lipid accumulation in AMs, exacerbated alveolar proteinosis, increased lung opacities on imaging, and significantly deteriorated pulmonary function in mice. Similarly, DTX4 depletion in primary AMs impaired cholesterol efflux and promoted intracellular lipid deposition. In contrast, AM-specific overexpression of DTX4 markedly alleviated lipid accumulation, mitigated alveolar proteinosis, restored lung densities on computed tomography, and rescued pulmonary function in Csf2ra-/-mice, a model of PAP. Mechanistically, DTX4 deficiency downregulated PPAR-γ expression, driving foamy AM formation. Notably, the regulatory function of DTX4 in lipid homeostasis was partially mediated by PPAR-γ but independent of its canonical E3 ubiquitin ligase activity. Collectively, our findings establish DTX4 as a central orchestrator of AM cholesterol efflux and surfactant homeostasis, positioning it as a promising therapeutic target for PAP and a potential paradigm for cholesterol dysregulation in related disorders.

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A Genome-Wide CRISPR Screen Identifies DTX4 Modulating Alveolar Macrophage Cholesterol Efflux in Pulmonary Alveolar Proteinosis [RNA-seq 1]

2026-04-23 | GSE299689 | GEO

A Genome-Wide CRISPR Screen Identifies DTX4 Modulating Alveolar Macrophage Cholesterol Efflux in Pulmonary Alveolar Proteinosis [RNA-seq II]

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Proteogenomic analysis of anti granulocyte macrophage colony- stimulating factor (GM-CSF) autoantibodies in the peripheral blood of a patient with autoimmune pulmonary alveolar proteinosis

Project description:In this study, we first analyzed the serological repertoire of anti-GM-CSF autoantibodies in a patients serum with autoimmune pulmonary alveolar proteinosis, using the trapped ion mobility spectrometry coupled with quadrupole time-of-flight (timsTOF) Pro mass spectrometry. The timsTOF Pro identified peptides that partially matched sequences in up to 96 percent of cDNA clones. Complementarity-determining region 3 (CDR3), is the most diverse in terms of sequence, was fully and partially detected in nine and 132 clones, respectively. Moreover, we confirmed one unique framework region 4 (FR4) and at least three unique across CDR3 to FR4 peptides via de novo peptide sequencing. This new technology may thus permit the comprehensive identification of polyclonal autoantibody structure.

2020-09-12 | PXD015420 | JPOST Repository