Lymphangioleiomyomatosis (LAM) is a rare lung disease of women, causing cystic remodelling of the lung and progressive respiratory failure. The cellular composition, microenvironment and cellular interactions within the LAM lesion remain unclear. To facilitate data sharing and collaborative LAM research, we performed an integrative analysis of single-cell data compiled from lung, uterus and kidney of patients with LAM from three research centres and developed an LAM Cell Atlas (LCA) Web-Portal. The LCA offers a variety of interactive options for investigators to search, visualise and reanalyse comprehensive single-cell multiomics data sets to reveal dysregulated genetic programmes at transcriptomic, epigenomic and cell–cell connectome levels.
- rare lung diseases
- idiopathic pulmonary fibrosis
- lung cancer
- cystic fibrosis
- pulmonary lymphoma
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Contributors FM and YX conceived and designed the experiments. EK coordinated tissue collection and organisation of fresh tissue. KW-B processed, evaluated and selected human lung tissues for single-cell RNA-seq/ATAC-seq and provided LAM tissue images. AKP and JY provided LAM research related images. MG, YW, AW and YX designed and performed lymphangioleiomyomatosis (LAM) single-cell RNA/ATAC analysis and ligand-receptor interactome analyses for UC/CCHMC data cohort and three-cohort data integration. YT, DK and EPH contributed LAM lung single-cell RNA-seq from BWH data cohort. KO and VK contributed LAM lung single-cell RNA-seq of UPenn data cohort. YD, MG and YX designed web portal; YD developed web application. NG and FM coordinated tissue collection and regulatory issues; and provided feedback on LCA construction. YX, YD and FM wrote the manuscript. All authors contributed to the manuscript writing and editing. All the authors read and approved the final manuscript.
Funding Supported by the LAM Foundation (LAM0141E01-20 to JY, and YX; LAM0138E01-19 to AKP and YX; LAM0142C01-20 to YT; LAM0131PB07-18 to FM and AKP) and the NHLBI (R01HL153045 to YX, and JY; R01HL141462, R01HL158737, and R01HL151647 to VK; U01HL148856 to YX; LungMAP2 pilot award to MG; U54HL127672 and U01HL131755-03 to FM; U01HL131022 to EPH, FM, VK).
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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