Lymphangioleiomyomatosis (LAM) Cell Atlas

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.

[1]  N. Gupta,et al.  Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. , 2021, The Lancet. Respiratory medicine.

[2]  Jilly F. Evans,et al.  mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline , 2020, Nature Communications.

[3]  D. Kwiatkowski,et al.  mTORC1 hyperactivation in lymphangioleiomyomatosis leads to ACE2 upregulation in type II pneumocytes: implications for COVID-19 , 2020, European Respiratory Journal.

[4]  D. Kwiatkowski,et al.  Single Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell. , 2020, American journal of respiratory and critical care medicine.

[5]  H. Harashima,et al.  Heterogeneity of tumor endothelial cells and drug delivery. , 2016, Advanced Drug Delivery Reviews.

[6]  W. Travis,et al.  Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. , 2012, American journal of respiratory and critical care medicine.

[7]  C. Burger Efficacy and safety of sirolimus in lymphangioleiomyomatosis. , 2011, The New England journal of medicine.

[8]  E. Henske,et al.  The spectrum of mutations in TSC1 and TSC2 in women with tuberous sclerosis and lymphangiomyomatosis. , 2001, American journal of respiratory and critical care medicine.

[9]  E. Henske,et al.  Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[10]  E. Henske,et al.  Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis. , 1998, American journal of human genetics.