Research graph
References from Current insight into the molecular mechanisms of lymphangioleiomyomatosis. Local targets link to admitted publications; unresolved targets remain external evidence.
Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management
10.1016/s2213-2600(21)00228-9 · 2021 · External reference
Updated prevalence of lymphangioleiomyomatosis in Europe
10.1164/rccm.202310-1736le · 2024 · External reference
Lymphangioleiomyomatosis: no longer ultra-rare
10.1164/rccm.202312-2254ed · 2024 · External reference
Lymphangioleiomyomatosis–a wolf in sheep’s clothing
10.1172/jci58709 · 2012 · External reference
Single-cell transcriptomic analysis identifies a unique pulmonary lymphangioleiomyomatosis cell
10.1164/rccm.201912-2445oc · 2020 · External reference
Tuberin regulates p70 S6 kinase activation and ribosomal protein S6 phosphorylation: a role for the TSC2 tumor suppressor gene in pulmonary lymphangioleiomyomatosis (LAM)
10.1074/jbc.m202678200 · 2002 · External reference
Efficacy and safety of sirolimus in lymphangioleiomyomatosis
10.1056/nejmoa1100391 · 2011 · External reference
Sustained effects of sirolimus on lung function and cystic lung lesions in lymphangioleiomyomatosis
10.1164/rccm.201405-0918oc · 2014 · External reference
Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial
10.1016/s2213-2600(13)70090-0 · 2013 · External reference
The tuberous sclerosis complex: balancing proliferation and survival
10.1042/bst0390466 · 2011 · External reference
Lymphangioleiomyomatosis (LAM): molecular insights lead to targeted therapies
10.1016/j.rmed.2010.03.017 · 2010 · External reference
TSC2 integrates Wnt and energy signals via a coordinated phosphorylation by AMPK and GSK3 to regulate cell growth
10.1016/j.cell.2006.06.055 · 2006 · External reference
Identification of 54 large deletions/duplications in TSC1 and TSC2 using MLPA, and genotype-phenotype correlations
10.1007/s00439-006-0308-9 · 2007 · External reference
Genomic rearrangements in sporadic lymphangioleiomyomatosis: an evolving genetic story
10.1038/modpathol.2017.52 · 2017 · External reference
Generalised mosaicism for TSC2 mutation in isolated lymphangioleiomyomatosis
10.1183/13993003.00938-2019 · 2019 · External reference
Sporadic lymphangioleiomyomatosis in a man with somatic mosaicism of TSC2 mutations, a case report
10.1093/qjmed/hcad235 · 2024 · External reference
A genome-wide association study implicates NR2F2 in lymphangioleiomyomatosis pathogenesis
10.1183/13993003.00329-2019 · 2019 · External reference
Gene mutations in sporadic lymphangioleiomyomatosis and genotype–phenotype correlation analysis
10.1186/s12890-022-02154-0 · 2022 · External reference
The codon 72 TP53 polymorphism contributes to TSC tumorigenesis through the notch–nodal axis
10.1158/1541-7786.mcr-18-1292 · 2019 · External reference
Heterogeneity and cancer-related features in lymphangioleiomyomatosis cells and tissue
10.1158/1541-7786.mcr-21-0220 · 2021 · External reference
Single-cell multiomic analysis identifies a HOX-PBX gene network regulating the survival of lymphangioleiomyomatosis cells
10.1126/sciadv.adf8549 · 2023 · External reference
Tuberin regulates E-cadherin localization: implications in epithelial-mesenchymal transition
10.2353/ajpath.2010.090233 · 2010 · External reference
Tissue-engineered disease modeling of lymphangioleiomyomatosis exposes a therapeutic vulnerability to HDAC inhibition
10.1002/advs.202302611 · 2023 · External reference
Extracellular matrix deposition drives disease progression and reduces rapamycin response in LAM
10.1183/13993003.00492-2025 · External reference
Targeting fibroblast–endothelial cell interactions in LAM pathogenesis using 3D spheroid models and spatial transcriptomics
10.1172/jci.insight.187899 · 2025 · External reference
Lost in translation: a neglected mTOR target for lymphangioleiomyomatosis
10.1183/16000617.0100-2023 · 2023 · External reference
Rapamycin-insensitive up-regulation of MMP2 and other genes in tuberous sclerosis complex 2–deficient lymphangioleiomyomatosis-like cells
10.1165/rcmb.2009-0050oc · 2010 · External reference
αB-crystallin/HSPB2 is critical for hyperactive mTOR-induced cardiomyopathy
10.1002/jcp.30465 · 2021 · External reference
TSC2 regulates lysosome biogenesis via a non-canonical RAGC and TFEB-dependent mechanism
10.1038/s41467-021-24499-6 · 2021 · External reference
TFEB drives mTORC1 hyperactivation and kidney disease in tuberous sclerosis complex
10.1038/s41467-023-44229-4 · 2024 · External reference
CIB2 regulates mTORC1 signaling and is essential for autophagy and visual function
10.1038/s41467-021-24056-1 · 2021 · External reference
Rapamycin-upregulated miR-29b promotes mTORC1-hyperactive cell growth in TSC2-deficient cells by downregulating tumor suppressor retinoic acid receptor β (RARβ)
10.1038/s41388-019-0957-5 · 2019 · External reference
Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts
10.1136/thoraxjnl-2020-214987 · 2020 · External reference
Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous estrogens
10.1378/chest.91.5.782 · 1987 · External reference
Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment
10.1136/thorax.57.12.1085 · 2002 · External reference
Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways
10.1152/ajplung.00204.2003 · 2004 · External reference
Estrogen promotes the survival and pulmonary metastasis of tuberin-null cells
10.1073/pnas.0810790106 · 2009 · External reference
Integration of mTOR and estrogen–ERK2 signaling in lymphangioleiomyomatosis pathogenesis
10.1073/pnas.1309110110 · 2013 · External reference
mTOR/S6K1 and MAPK/RSK signaling pathways coordinately regulate estrogen receptor alpha serine 167 phosphorylation
10.1016/j.febslet.2009.11.041 · 2010 · External reference
mTORC1 directly phosphorylates and activates ERα upon estrogen stimulation
10.1038/onc.2015.414 · 2016 · External reference
Estradiol promotes pentose phosphate pathway addiction and cell survival via reactivation of Akt in mTORC1 hyperactive cells
10.1038/cddis.2014.204 · 2014 · External reference
Histamine signaling and metabolism identify potential biomarkers and therapies for lymphangioleiomyomatosis
10.15252/emmm.202113929 · 2021 · External reference
Mast-cell tryptase release contributes to disease progression in lymphangioleiomyomatosis
10.1164/rccm.202007-2854oc · 2021 · External reference
Mammalian target of rapamycin regulates murine and human cell differentiation through STAT3/p63/jagged/notch Cascade
10.1172/jci37964 · 2010 · External reference
Signal transducer and activator of transcription 3 is required for abnormal proliferation and survival of TSC2-deficient cells: relevance to pulmonary lymphangioleiomyomatosis
10.1124/mol.109.057042 · 2009 · External reference
Notch transactivates RHEB to maintain the multipotency of TSC-null cells
10.1038/s41467-017-01845-1 · 2017 · External reference
Enhanced gαq signaling in TSC2-deficient cells is required for their neoplastic behavior
10.1165/rcmb.2024-0111oc · 2025 · External reference
miR‑124 targets retinoid X receptor α to reduce growth of TSC2‑deficient lymphangioleiomyomatosis
10.3892/or.2018.6916 · 2019 · External reference
Vps34-mediated macropinocytosis in tuberous sclerosis complex 2-deficient cells supports tumorigenesis
10.1038/s41598-018-32256-x · 2018 · External reference
Interleukin-6 mediates PSAT1 expression and serine metabolism in TSC2-deficient cells
10.1073/pnas.2101268118 · 2021 · External reference
Glucose addiction of TSC-null cells is caused by failed mTORC1-dependent balancing of metabolic demand with supply
10.1016/j.molcel.2010.05.007 · 2010 · External reference
Activation of a metabolic gene regulatory network downstream of mTOR complex 1
10.1016/j.molcel.2010.06.022 · 2010 · External reference
Glutaredoxin-1 promotes lymphangioleiomyomatosis progression through inhibiting Bim-mediated apoptosis via COX2/PGE2/ERK pathway
10.1002/ctm2.1333 · 2023 · External reference
SRC kinase is a novel therapeutic target in lymphangioleiomyomatosis
10.1158/0008-5472.can-13-1256 · 2014 · External reference
Osteoprotegerin contributes to the metastatic potential of cells with a dysfunctional TSC2 tumor-suppressor gene
10.1016/j.ajpath.2013.05.024 · 2013 · External reference
TSC2 modulates cell adhesion and migration via integrin-α1β1
10.1152/ajplung.00414.2011 · 2012 · External reference
Cathepsin-K expression in pulmonary lymphangioleiomyomatosis
10.1038/modpathol.2008.189 · 2009 · External reference
Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis
10.5858/2000-124-0267-rfaomm · 2000 · External reference
Matrix metalloproteinases in blood from patients with LAM
10.1016/j.rmed.2008.07.017 · 2009 · External reference
Differential modulation of matrix metalloproteinases-2 and -7 in LAM/TSC cells
10.3390/biomedicines9121760 · 2021 · External reference
Exploring the role of matrix metalloproteinases as biomarkers in sporadic lymphangioleiomyomatosis and tuberous sclerosis complex: a pilot study
10.3389/fmed.2021.605909 · 2021 · External reference
Small extracellular vesicles and induction of epithelial to mesenchymal transition in lymphangioleiomyomatosis
10.1183/13993003.02455-2024 · 2025 · External reference
Rheb activates AMPK and reduces p27Kip1 levels in Tsc2-null cells via mTORC1-independent mechanisms: implications for cell proliferation and tumorigenesis
10.1038/onc.2010.393 · 2010 · External reference
Lymphangioleiomyomatosis: a monogenic model of malignancy
10.1146/annurev-med-050715-104245 · 2017 · External reference
The lymphangioleiomyomatosis lung cell and its human cell models
10.1165/rcmb.2017-0403tr · 2018 · External reference
Phenotypic characterization of disseminated cells with TSC2 loss of heterozygosity in patients with lymphangioleiomyomatosis
10.1164/rccm.201003-0489oc · 2010 · External reference
Recurrent lymphangiomyomatosis after transplantation
10.1164/rccm.200208-969oc · 2003 · External reference
Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis
10.1073/pnas.0407971101 · 2004 · External reference
Uterine-specific loss of Tsc2 leads to myometrial tumors in both the uterus and lungs
10.1210/me.2013-1059 · 2013 · External reference
Associations of VEGF-D levels with clinical manifestations in lymphangioleiomyomatosis: a cross-sectional analysis of 631 cases
10.1186/s13023-025-03802-4 · 2025 · External reference
Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement
10.1378/chest.08-1160 · 2009 · External reference
Lymphatic involvement in lymphangioleiomyomatosis
10.1196/annals.1413.018 · 2008 · External reference
Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis
10.1183/09059180.00009311 · 2012 · External reference
Lymphatic endothelial differentiation in pulmonary lymphangioleiomyomatosis cells
10.1369/0022155413489311 · 2013 · External reference
Long-term effect of sirolimus on serum vascular endothelial growth factor D levels in patients with lymphangioleiomyomatosis
10.1016/j.chest.2017.05.012 · 2018 · External reference
mTORC1 upregulates B7-H3/CD276 to inhibit antitumor T cells and drive tumor immune evasion
10.1038/s41467-023-36881-7 · 2023 · External reference
NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline
10.1172/jci.insight.87270 · 2016 · External reference
Chemokine-enhanced chemotaxis of lymphangioleiomyomatosis cells with mutations in the tumor suppressor TSC2 gene
10.4049/jimmunol.182.3.1270 · 2009 · External reference
MCP-1 overexpressed in tuberous sclerosis lesions acts as a paracrine factor for tumor development
10.1084/jem.20042469 · 2005 · External reference
Modulation of infiltrating CD206-positive macrophages restricts progression of pulmonary lymphangioleiomyomatosis (LAM)
10.1183/13993003.00084-2025 · 2025 · External reference
Repression of TSC1/TSC2 mediated by MeCP2 regulates human embryo lung fibroblast cell differentiation and proliferation
10.1016/j.ijbiomac.2016.12.062 · 2017 · External reference
