Lymphatic System Development and Function.

Lymphatic development Lymphatic malformation Organ-specific lymphatic function

Journal

Current cardiology reports
ISSN: 1534-3170
Titre abrégé: Curr Cardiol Rep
Pays: United States
ID NLM: 100888969

Informations de publication

Date de publication:
22 Aug 2024
Historique:
accepted: 13 08 2024
medline: 22 8 2024
pubmed: 22 8 2024
entrez: 22 8 2024
Statut: aheadofprint

Résumé

This review delves into recent advancements in understanding generalized and organ-specific lymphatic development. It emphasizes the distinct characteristics and critical anomalies that can impair lymphatic function. By exploring developmental mechanisms, the review seeks to illuminate the profound impact of lymphatic malformations on overall health and disease progression. The introduction of genome sequencing, single-cell transcriptomic analysis, and advanced imaging technologies has significantly enhanced our ability to identify and characterize developmental defects within the lymphatic system. As a result, a wide range of lymphatic anomalies have been uncovered, spanning from congenital abnormalities present at birth to conditions that can become life-threatening in adulthood. Additionally, recent research highlights the heterogeneity of lymphatics, revealing organ-specific developmental pathways, unique molecular markers, and specialized physiological functions specific to each organ. A deeper understanding of the unique characteristics of lymphatic cell populations in an organ-specific context is essential for guiding future research into lymphatic disease processes. An integrated approach to translational research could revolutionize personalized medicine, where treatments are precisely tailored to individual lymphatic profiles, enhancing effectiveness and minimizing side effects.

Identifiants

pubmed: 39172295
doi: 10.1007/s11886-024-02120-8
pii: 10.1007/s11886-024-02120-8
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : NHLBI NIH HHS
ID : R01HL141377
Pays : United States

Informations de copyright

© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

Références

Oliver G, Kipnis J, Randolph GJ, Harvey NL. The Lymphatic Vasculature in the 21st Century: Novel Functional Roles in Homeostasis and Disease. Cell. 2020;182(2):270–96. https://doi.org/10.1016/j.cell.2020.06.039 .
doi: 10.1016/j.cell.2020.06.039 pubmed: 32707093 pmcid: 7392116
Petrova TV, Koh GY. Biological functions of lymphatic vessels. Science. 2020;369(6500):eaax4063. https://doi.org/10.1126/science.aax4063 .
doi: 10.1126/science.aax4063 pubmed: 32646971
Makinen T, Boon LM, Vikkula M, Alitalo K. Lymphatic Malformations: Genetics, Mechanisms and Therapeutic Strategies. Circ Res. 2021;129(1):136–54. https://doi.org/10.1161/CIRCRESAHA.121.318142 .
doi: 10.1161/CIRCRESAHA.121.318142 pubmed: 34166072
Desai SB, Iacobas I, Rockson SG. Lymphatic Development and Implications for Diagnosis and Therapy. Lymphat Res Biol. 2021;19(1):31–5. https://doi.org/10.1089/lrb.2020.0123 .
doi: 10.1089/lrb.2020.0123 pubmed: 33625891
Singhal D, Borner K, Chaikof EL, Detmar M, Hollmen M, Iliff JJ, et al. Mapping the lymphatic system across body scales and expertise domains: A report from the 2021 National Heart, Lung, and Blood Institute workshop at the Boston Lymphatic Symposium. Front Physiol. 2023;14:1099403. https://doi.org/10.3389/fphys.2023.1099403 .
doi: 10.3389/fphys.2023.1099403 pubmed: 36814475 pmcid: 9939837
Rockson SG, Rivera KK. Estimating the population burden of lymphedema. Ann N Y Acad Sci. 2008;1131:147–54. https://doi.org/10.1196/annals.1413.014 .
doi: 10.1196/annals.1413.014 pubmed: 18519968
Jung R, Trivedi CM. Congenital Vascular and Lymphatic Diseases. Circ Res. 2024;135(1):159–73. https://doi.org/10.1161/CIRCRESAHA.124.323181 .
doi: 10.1161/CIRCRESAHA.124.323181 pubmed: 38900856
Mehrara BJ, Radtke AJ, Randolph GJ, Wachter BT, Greenwel P, Rovira, II, et al. The emerging importance of lymphatics in health and disease: an NIH workshop report. J Clin Invest. 2023;133(17). https://doi.org/10.1172/JCI171582 .
Janardhan HP, Saheera S, Jung R, Trivedi CM. Vascular and Lymphatic Malformations: Perspectives From Human and Vertebrate Studies. Circ Res. 2021;129(1):131–5. https://doi.org/10.1161/CIRCRESAHA.121.319587 .
doi: 10.1161/CIRCRESAHA.121.319587 pubmed: 34166069 pmcid: 8238353
Radtke AJ, Lukacs JM, Praskievicz NE, Muratoglu SC, Rovira II, Galis ZS. The centuries long pursuit to map the human lymphatic system. Nat Med. 2022;28(8):1518–20. https://doi.org/10.1038/s41591-022-01865-5 .
doi: 10.1038/s41591-022-01865-5 pubmed: 35681105
Sabin FR. The lymphatic system in human embryos, with a consideration of the morphology of the system as a whole. Am J Anat. 1909;9(1):43–91. https://doi.org/10.1002/aja.1000090104 .
doi: 10.1002/aja.1000090104
Huntington GS, McClure CFW. The anatomy and development of the jugular lymph sacs in the domestic cat (Felis domestica). Am J Anat. 1910;10(1):177–312. https://doi.org/10.1002/aja.1000100108 .
doi: 10.1002/aja.1000100108
Wigle JT, Oliver G. Prox1 function is required for the development of the murine lymphatic system. Cell. 1999;98(6):769–78. https://doi.org/10.1016/s0092-8674(00)81511-1 .
doi: 10.1016/s0092-8674(00)81511-1 pubmed: 10499794
Stone OA, Stainier DYR. Paraxial Mesoderm Is the Major Source of Lymphatic Endothelium. Dev Cell. 2019;50(2):247-55 e3. https://doi.org/10.1016/j.devcel.2019.04.034 .
doi: 10.1016/j.devcel.2019.04.034 pubmed: 31130354 pmcid: 6658618
Joukov V, Pajusola K, Kaipainen A, Chilov D, Lahtinen I, Kukk E, et al. A novel vascular endothelial growth factor, VEGF-C, is a ligand for the Flt4 (VEGFR-3) and KDR (VEGFR-2) receptor tyrosine kinases. EMBO J. 1996;15(2):290–8.
doi: 10.1002/j.1460-2075.1996.tb00359.x pubmed: 8617204 pmcid: 449944
Srinivasan RS, Dillard ME, Lagutin OV, Lin FJ, Tsai S, Tsai MJ, et al. Lineage tracing demonstrates the venous origin of the mammalian lymphatic vasculature. Genes Dev. 2007;21(19):2422–32. https://doi.org/10.1101/gad.1588407 .
doi: 10.1101/gad.1588407 pubmed: 17908929 pmcid: 1993873
Kaipainen A, Korhonen J, Mustonen T, van Hinsbergh VW, Fang GH, Dumont D, et al. Expression of the fms-like tyrosine kinase 4 gene becomes restricted to lymphatic endothelium during development. Proc Natl Acad Sci U S A. 1995;92(8):3566–70. https://doi.org/10.1073/pnas.92.8.3566 .
doi: 10.1073/pnas.92.8.3566 pubmed: 7724599 pmcid: 42208
Jafree DJ, Long DA, Scambler PJ, Ruhrberg C. Mechanisms and cell lineages in lymphatic vascular development. Angiogenesis. 2021;24(2):271–88. https://doi.org/10.1007/s10456-021-09784-8 .
doi: 10.1007/s10456-021-09784-8 pubmed: 33825109 pmcid: 8205918
Yamaguchi S, Minamide N, Imai H, Ikeda T, Watanabe M, Imanaka-Yoshida K, et al. The development of early human lymphatic vessels as characterized by lymphatic endothelial markers. EMBO J. 2024;43(5):868–85. https://doi.org/10.1038/s44318-024-00045-0 .
doi: 10.1038/s44318-024-00045-0 pubmed: 38351385 pmcid: 10907744
Hess PR, Rawnsley DR, Jakus Z, Yang Y, Sweet DT, Fu J, et al. Platelets mediate lymphovenous hemostasis to maintain blood-lymphatic separation throughout life. J Clin Invest. 2014;124(1):273–84. https://doi.org/10.1172/JCI70422 .
doi: 10.1172/JCI70422 pubmed: 24292710
Srinivasan RS, Oliver G. Prox1 dosage controls the number of lymphatic endothelial cell progenitors and the formation of the lymphovenous valves. Genes Dev. 2011;25(20):2187–97. https://doi.org/10.1101/gad.16974811 .
doi: 10.1101/gad.16974811 pubmed: 22012621 pmcid: 3205588
O’Hagan LA, Windsor JA, Phillips ARJ, Itkin M, Russell PS, Mirjalili SA. Anatomy of the lymphovenous valve of the thoracic duct in humans. J Anat. 2020;236(6):1146–53. https://doi.org/10.1111/joa.13167 .
doi: 10.1111/joa.13167 pubmed: 32103496 pmcid: 7219621
Geng X, Srinivasan RS. Correlative Fluorescence and Scanning Electron Microscopy to Study Lymphovenous Valve Development. Methods Mol Biol. 2018;1846:85–96. https://doi.org/10.1007/978-1-4939-8712-2_6 .
doi: 10.1007/978-1-4939-8712-2_6 pubmed: 30242754
O’Hagan LA, Windsor JA, Itkin M, Russell PS, Phillips ARJ, Mirjalili SA. The Lymphovenous Junction of the Thoracic Duct: A Systematic Review of its Structural and Functional Anatomy. Lymphat Res Biol. 2021;19(3):215–22. https://doi.org/10.1089/lrb.2020.0010 .
doi: 10.1089/lrb.2020.0010 pubmed: 33232643
Geng X, Cha B, Mahamud MR, Lim KC, Silasi-Mansat R, Uddin MKM, et al. Multiple mouse models of primary lymphedema exhibit distinct defects in lymphovenous valve development. Dev Biol. 2016;409(1):218–33. https://doi.org/10.1016/j.ydbio.2015.10.022 .
doi: 10.1016/j.ydbio.2015.10.022 pubmed: 26542011
Janardhan HP, Milstone ZJ, Shin M, Lawson ND, Keaney JF Jr, Trivedi CM. Hdac3 regulates lymphovenous and lymphatic valve formation. J Clin Invest. 2017;127(11):4193–206. https://doi.org/10.1172/JCI92852 .
doi: 10.1172/JCI92852 pubmed: 29035278 pmcid: 5663362
Janardhan HP, Trivedi CM. Establishment and maintenance of blood-lymph separation. Cell Mol Life Sci. 2019;76(10):1865–76. https://doi.org/10.1007/s00018-019-03042-3 .
doi: 10.1007/s00018-019-03042-3 pubmed: 30758642 pmcid: 6482084
Ohhashi T, Azuma T, Sakaguchi M. Active and passive mechanical characteristics of bovine mesenteric lymphatics. Am J Physiol. 1980;239(1):H88-95. https://doi.org/10.1152/ajpheart.1980.239.1.H88 .
doi: 10.1152/ajpheart.1980.239.1.H88 pubmed: 7396023
Olszewski WL. Contractility patterns of normal and pathologically changed human lymphatics. Ann N Y Acad Sci. 2002;979:52–63. https://doi.org/10.1111/j.1749-6632.2002.tb04867.x .
doi: 10.1111/j.1749-6632.2002.tb04867.x pubmed: 12543716
Mellor RH, Brice G, Stanton AW, French J, Smith A, Jeffery S, et al. Mutations in FOXC2 are strongly associated with primary valve failure in veins of the lower limb. Circulation. 2007;115(14):1912–20. https://doi.org/10.1161/CIRCULATIONAHA.106.675348 .
doi: 10.1161/CIRCULATIONAHA.106.675348 pubmed: 17372167
Petrova TV, Karpanen T, Norrmen C, Mellor R, Tamakoshi T, Finegold D, et al. Defective valves and abnormal mural cell recruitment underlie lymphatic vascular failure in lymphedema distichiasis. Nat Med. 2004;10(9):974–81. https://doi.org/10.1038/nm1094 .
doi: 10.1038/nm1094 pubmed: 15322537
Norrmen C, Ivanov KI, Cheng J, Zangger N, Delorenzi M, Jaquet M, et al. FOXC2 controls formation and maturation of lymphatic collecting vessels through cooperation with NFATc1. J Cell Biol. 2009;185(3):439–57. https://doi.org/10.1083/jcb.200901104 .
doi: 10.1083/jcb.200901104 pubmed: 19398761 pmcid: 2700385
Sabine A, Agalarov Y, Maby-El Hajjami H, Jaquet M, Hagerling R, Pollmann C, et al. Mechanotransduction, PROX1, and FOXC2 cooperate to control connexin37 and calcineurin during lymphatic-valve formation. Dev Cell. 2012;22(2):430–45. https://doi.org/10.1016/j.devcel.2011.12.020 .
doi: 10.1016/j.devcel.2011.12.020 pubmed: 22306086
Gonzalez-Loyola A, Petrova TV. Development and aging of the lymphatic vascular system. Adv Drug Deliv Rev. 2021;169:63–78. https://doi.org/10.1016/j.addr.2020.12.005 .
doi: 10.1016/j.addr.2020.12.005 pubmed: 33316347
Bernier-Latmani J, Gonzalez-Loyola A, Petrova TV. Mechanisms and functions of intestinal vascular specialization. J Exp Med. 2024;221(1):e20222008. https://doi.org/10.1084/jem.20222008 .
doi: 10.1084/jem.20222008 pubmed: 38051275
Davis MJ, Zawieja SD, Yang Y. Developmental progression of lymphatic valve morphology and function. Front Cell Dev Biol. 2024;12:1331291. https://doi.org/10.3389/fcell.2024.1331291 .
doi: 10.3389/fcell.2024.1331291 pubmed: 38450249 pmcid: 10915029
Kanady JD, Dellinger MT, Munger SJ, Witte MH, Simon AM. Connexin37 and Connexin43 deficiencies in mice disrupt lymphatic valve development and result in lymphatic disorders including lymphedema and chylothorax. Dev Biol. 2011;354(2):253–66. https://doi.org/10.1016/j.ydbio.2011.04.004 .
doi: 10.1016/j.ydbio.2011.04.004 pubmed: 21515254 pmcid: 3134316
Kanady JD, Munger SJ, Witte MH, Simon AM. Combining Foxc2 and Connexin37 deletions in mice leads to severe defects in lymphatic vascular growth and remodeling. Dev Biol. 2015;405(1):33–46. https://doi.org/10.1016/j.ydbio.2015.06.004 .
doi: 10.1016/j.ydbio.2015.06.004 pubmed: 26079578 pmcid: 4529811
Sweet DT, Jimenez JM, Chang J, Hess PR, Mericko-Ishizuka P, Fu J, et al. Lymph flow regulates collecting lymphatic vessel maturation in vivo. J Clin Invest. 2015;125(8):2995–3007. https://doi.org/10.1172/JCI79386 .
doi: 10.1172/JCI79386 pubmed: 26214523 pmcid: 4563745
Bazigou E, Xie S, Chen C, Weston A, Miura N, Sorokin L, et al. Integrin-alpha9 is required for fibronectin matrix assembly during lymphatic valve morphogenesis. Dev Cell. 2009;17(2):175–86. https://doi.org/10.1016/j.devcel.2009.06.017 .
doi: 10.1016/j.devcel.2009.06.017 pubmed: 19686679 pmcid: 2747264
Kazenwadel J, Betterman KL, Chong CE, Stokes PH, Lee YK, Secker GA, et al. GATA2 is required for lymphatic vessel valve development and maintenance. J Clin Invest. 2015;125(8):2979–94. https://doi.org/10.1172/JCI78888 .
doi: 10.1172/JCI78888 pubmed: 26214525 pmcid: 4563742
Welsh JD, Kahn ML, Sweet DT. Lymphovenous hemostasis and the role of platelets in regulating lymphatic flow and lymphatic vessel maturation. Blood. 2016;128(9):1169–73. https://doi.org/10.1182/blood-2016-04-636415 .
doi: 10.1182/blood-2016-04-636415 pubmed: 27385789
Kazenwadel J, Secker GA, Liu YJ, Rosenfeld JA, Wildin RS, Cuellar-Rodriguez J, et al. Loss-of-function germline GATA2 mutations in patients with MDS/AML or MonoMAC syndrome and primary lymphedema reveal a key role for GATA2 in the lymphatic vasculature. Blood. 2012;119(5):1283–91. https://doi.org/10.1182/blood-2011-08-374363 .
doi: 10.1182/blood-2011-08-374363 pubmed: 22147895 pmcid: 3277359
Mahamud MR, Geng X, Chen L, Ahmed Z, Ho Y, Srinivasan RS. GATA2 regulates blood/lymph separation in a platelet-dependent and lymphovenous valve-independent manner. Microcirculation. 2023;30(2–3):e12787. https://doi.org/10.1111/micc.12787 .
doi: 10.1111/micc.12787 pubmed: 36197446
Spinner MA, Sanchez LA, Hsu AP, Shaw PA, Zerbe CS, Calvo KR, et al. GATA2 deficiency: a protean disorder of hematopoiesis, lymphatics, and immunity. Blood. 2014;123(6):809–21. https://doi.org/10.1182/blood-2013-07-515528 .
doi: 10.1182/blood-2013-07-515528 pubmed: 24227816 pmcid: 3916876
Ostergaard P, Simpson MA, Connell FC, Steward CG, Brice G, Woollard WJ, et al. Mutations in GATA2 cause primary lymphedema associated with a predisposition to acute myeloid leukemia (Emberger syndrome). Nat Genet. 2011;43(10):929–31. https://doi.org/10.1038/ng.923 .
doi: 10.1038/ng.923 pubmed: 21892158
Geng X, Ho YC, Srinivasan RS. Biochemical and mechanical signals in the lymphatic vasculature. Cell Mol Life Sci. 2021;78(16):5903–23. https://doi.org/10.1007/s00018-021-03886-8 .
doi: 10.1007/s00018-021-03886-8 pubmed: 34240226 pmcid: 11072415
Cha B, Geng X, Mahamud MR, Zhang JY, Chen L, Kim W, et al. Complementary Wnt Sources Regulate Lymphatic Vascular Development via PROX1-Dependent Wnt/beta-Catenin Signaling. Cell Rep. 2018;25(3):571-84 e5. https://doi.org/10.1016/j.celrep.2018.09.049 .
doi: 10.1016/j.celrep.2018.09.049 pubmed: 30332639 pmcid: 6264919
Baeyens N, Mulligan-Kehoe MJ, Corti F, Simon DD, Ross TD, Rhodes JM, et al. Syndecan 4 is required for endothelial alignment in flow and atheroprotective signaling. Proc Natl Acad Sci U S A. 2014;111(48):17308–13. https://doi.org/10.1073/pnas.1413725111 .
doi: 10.1073/pnas.1413725111 pubmed: 25404299 pmcid: 4260558
Baeyens N, Nicoli S, Coon BG, Ross TD, Van den Dries K, Han J, et al. Vascular remodeling is governed by a VEGFR3-dependent fluid shear stress set point. Elife. 2015;4:e04645. https://doi.org/10.7554/eLife.04645 .
doi: 10.7554/eLife.04645 pubmed: 25643397 pmcid: 4337723
Coon BG, Baeyens N, Han J, Budatha M, Ross TD, Fang JS, et al. Intramembrane binding of VE-cadherin to VEGFR2 and VEGFR3 assembles the endothelial mechanosensory complex. J Cell Biol. 2015;208(7):975–86. https://doi.org/10.1083/jcb.201408103 .
doi: 10.1083/jcb.201408103 pubmed: 25800053 pmcid: 4384728
Tzima E, Irani-Tehrani M, Kiosses WB, Dejana E, Schultz DA, Engelhardt B, et al. A mechanosensory complex that mediates the endothelial cell response to fluid shear stress. Nature. 2005;437(7057):426–31. https://doi.org/10.1038/nature03952 .
doi: 10.1038/nature03952 pubmed: 16163360
Wang Y, Baeyens N, Corti F, Tanaka K, Fang JS, Zhang J, et al. Syndecan 4 controls lymphatic vasculature remodeling during mouse embryonic development. Development. 2016;143(23):4441–51. https://doi.org/10.1242/dev.140129 .
doi: 10.1242/dev.140129 pubmed: 27789626 pmcid: 5201046
Pujol F, Hodgson T, Martinez-Corral I, Prats AC, Devenport D, Takeichi M, et al. Dachsous1-Fat4 Signaling Controls Endothelial Cell Polarization During Lymphatic Valve Morphogenesis-Brief Report. Arterioscler Thromb Vasc Biol. 2017;37(9):1732–5. https://doi.org/10.1161/ATVBAHA.117.309818 .
doi: 10.1161/ATVBAHA.117.309818 pubmed: 28705793
Tatin F, Taddei A, Weston A, Fuchs E, Devenport D, Tissir F, et al. Planar cell polarity protein Celsr1 regulates endothelial adherens junctions and directed cell rearrangements during valve morphogenesis. Dev Cell. 2013;26(1):31–44. https://doi.org/10.1016/j.devcel.2013.05.015 .
doi: 10.1016/j.devcel.2013.05.015 pubmed: 23792146 pmcid: 3714594
Alders M, Al-Gazali L, Cordeiro I, Dallapiccola B, Garavelli L, Tuysuz B, et al. Hennekam syndrome can be caused by FAT4 mutations and be allelic to Van Maldergem syndrome. Hum Genet. 2014;133(9):1161–7. https://doi.org/10.1007/s00439-014-1456-y .
doi: 10.1007/s00439-014-1456-y pubmed: 24913602
Alders M, Hogan BM, Gjini E, Salehi F, Al-Gazali L, Hennekam EA, et al. Mutations in CCBE1 cause generalized lymph vessel dysplasia in humans. Nat Genet. 2009;41(12):1272–4. https://doi.org/10.1038/ng.484 .
doi: 10.1038/ng.484 pubmed: 19935664
Gonzalez-Garay ML, Aldrich MB, Rasmussen JC, Guilliod R, Lapinski PE, King PD, et al. A novel mutation in CELSR1 is associated with hereditary lymphedema. Vasc Cell. 2016;8:1. https://doi.org/10.1186/s13221-016-0035-5 .
doi: 10.1186/s13221-016-0035-5 pubmed: 26855770 pmcid: 4743364
Jung R, Janardhan HP, Trivedi CM. Cation Channelopathies: Novel Insights into Generalized Lymphatic Dysplasia. Circ Res. 2022;131(2):130–2. https://doi.org/10.1161/CIRCRESAHA.122.321400 .
doi: 10.1161/CIRCRESAHA.122.321400 pubmed: 35861738 pmcid: 9304756
Coste B, Mathur J, Schmidt M, Earley TJ, Ranade S, Petrus MJ, et al. Piezo1 and Piezo2 are essential components of distinct mechanically activated cation channels. Science. 2010;330(6000):55–60. https://doi.org/10.1126/science.1193270 .
doi: 10.1126/science.1193270 pubmed: 20813920 pmcid: 3062430
Choi D, Park E, Yu RP, Cooper MN, Cho IT, Choi J, et al. Piezo1-Regulated Mechanotransduction Controls Flow-Activated Lymphatic Expansion. Circ Res. 2022;131(2):e2–21. https://doi.org/10.1161/CIRCRESAHA.121.320565 .
doi: 10.1161/CIRCRESAHA.121.320565 pubmed: 35701867 pmcid: 9308715
Du J, Liu P, Zhou Y, Misener S, Sharma I, Leeaw P, et al. The mechanosensory channel PIEZO1 functions upstream of angiopoietin/TIE/FOXO1 signaling in lymphatic development. J Clin Invest. 2024;134(10). https://doi.org/10.1172/JCI176577 .
Choi D, Park E, Jung E, Cha B, Lee S, Yu J, et al. Piezo1 incorporates mechanical force signals into the genetic program that governs lymphatic valve development and maintenance. JCI Insight. 2019;4(5). https://doi.org/10.1172/jci.insight.125068 .
Nonomura K, Lukacs V, Sweet DT, Goddard LM, Kanie A, Whitwam T, et al. Mechanically activated ion channel PIEZO1 is required for lymphatic valve formation. Proc Natl Acad Sci U S A. 2018;115(50):12817–22. https://doi.org/10.1073/pnas.1817070115 .
doi: 10.1073/pnas.1817070115 pubmed: 30482854 pmcid: 6294938
Hussmann M, Schulte D, Weischer S, Carlantoni C, Nakajima H, Mochizuki N, et al. Svep1 is a binding ligand of Tie1 and affects specific aspects of facial lymphatic development in a Vegfc-independent manner. Elife. 2023;12:e82969. https://doi.org/10.7554/eLife.82969 .
doi: 10.7554/eLife.82969 pubmed: 37097004 pmcid: 10129328
Morooka N, Gui N, Ando K, Sako K, Fukumoto M, Hasegawa U, et al. Angpt1 binding to Tie1 regulates the signaling required for lymphatic vessel development in zebrafish. Development. 2024;151(10):dev202269. https://doi.org/10.1242/dev.202269 .
doi: 10.1242/dev.202269 pubmed: 38742432
Qu X, Zhou B, Scott BH. Tie1 is required for lymphatic valve and collecting vessel development. Dev Biol. 2015;399(1):117–28. https://doi.org/10.1016/j.ydbio.2014.12.021 .
doi: 10.1016/j.ydbio.2014.12.021 pubmed: 25576926 pmcid: 4374493
Muley A, Kim UhM, Salazar-De Simone G, Swaminathan B, James JM, Murtomaki A, et al. Unique functions for Notch4 in murine embryonic lymphangiogenesis. Angiogenesis. 2022;25(2):205–24. https://doi.org/10.1007/s10456-021-09822-5 .
doi: 10.1007/s10456-021-09822-5 pubmed: 34665379
Britto DD, He J, Misa JP, Chen W, Kakadia PM, Grimm L, et al. Plexin D1 negatively regulates zebrafish lymphatic development. Development. 2022;149(21):dev200560. https://doi.org/10.1242/dev.200560 .
doi: 10.1242/dev.200560 pubmed: 36205097 pmcid: 9720674
Bouvree K, Brunet I, Del Toro R, Gordon E, Prahst C, Cristofaro B, et al. Semaphorin3A, Neuropilin-1, and PlexinA1 are required for lymphatic valve formation. Circ Res. 2012;111(4):437–45. https://doi.org/10.1161/CIRCRESAHA.112.269316 .
doi: 10.1161/CIRCRESAHA.112.269316 pubmed: 22723296
Jurisic G, Maby-El Hajjami H, Karaman S, Ochsenbein AM, Alitalo A, Siddiqui SS, et al. An unexpected role of semaphorin3a-neuropilin-1 signaling in lymphatic vessel maturation and valve formation. Circ Res. 2012;111(4):426–36. https://doi.org/10.1161/CIRCRESAHA.112.269399 .
doi: 10.1161/CIRCRESAHA.112.269399 pubmed: 22723300 pmcid: 3572231
Levet S, Ciais D, Merdzhanova G, Mallet C, Zimmers TA, Lee SJ, et al. Bone morphogenetic protein 9 (BMP9) controls lymphatic vessel maturation and valve formation. Blood. 2013;122(4):598–607. https://doi.org/10.1182/blood-2012-12-472142 .
doi: 10.1182/blood-2012-12-472142 pubmed: 23741013 pmcid: 3724195
Murtomaki A, Uh MK, Kitajewski C, Zhao J, Nagasaki T, Shawber CJ, et al. Notch signaling functions in lymphatic valve formation. Development. 2014;141(12):2446–51. https://doi.org/10.1242/dev.101188 .
doi: 10.1242/dev.101188 pubmed: 24917500 pmcid: 4050693
Martin-Almedina S, Martinez-Corral I, Holdhus R, Vicente A, Fotiou E, Lin S, et al. EPHB4 kinase-inactivating mutations cause autosomal dominant lymphatic-related hydrops fetalis. J Clin Invest. 2016;126(8):3080–8. https://doi.org/10.1172/JCI85794 .
doi: 10.1172/JCI85794 pubmed: 27400125 pmcid: 4966301
Naiche LA, Villa SR, Kitajewski JK. Endothelial Cell Fate Determination: A Top Notch Job in Vascular Decision-Making. Cold Spring Harb Perspect Med. 2022;12(11):a041183. https://doi.org/10.1101/cshperspect.a041183 .
doi: 10.1101/cshperspect.a041183 pubmed: 35288401
Akangire G, Menden H, Xia S, Thiffault I, Ahmed A, Sampath V. EPHB4 Mutation Suppresses PROX1 Expression and Disrupts Lymphatic Development in Neonatal Hydrops. Pediatrics. 2022;149(3). https://doi.org/10.1542/peds.2021-053294 .
Makinen T, Adams RH, Bailey J, Lu Q, Ziemiecki A, Alitalo K, et al. PDZ interaction site in ephrinB2 is required for the remodeling of lymphatic vasculature. Genes Dev. 2005;19(3):397–410. https://doi.org/10.1101/gad.330105 .
doi: 10.1101/gad.330105 pubmed: 15687262 pmcid: 546518
Ma GC, Liu CS, Chang SP, Yeh KT, Ke YY, Chen TH, et al. A recurrent ITGA9 missense mutation in human fetuses with severe chylothorax: possible correlation with poor response to fetal therapy. Prenat Diagn. 2008;28(11):1057–63. https://doi.org/10.1002/pd.2130 .
doi: 10.1002/pd.2130 pubmed: 18973153
Rogerson D, Alkelai A, Giordano J, Pantrangi M, Hsiao MC, Nhan-Chang CL, et al. Investigation into the genetics of fetal congenital lymphatic anomalies. Prenat Diagn. 2023;43(6):703–16. https://doi.org/10.1002/pd.6345 .
doi: 10.1002/pd.6345 pubmed: 36959127 pmcid: 10330091
Danussi C, Del Bel BL, Pivetta E, Modica TM, Muro A, Wassermann B, et al. EMILIN1/alpha9beta1 integrin interaction is crucial in lymphatic valve formation and maintenance. Mol Cell Biol. 2013;33(22):4381–94. https://doi.org/10.1128/MCB.00872-13 .
doi: 10.1128/MCB.00872-13 pubmed: 24019067 pmcid: 3838180
Petrova TV, Koh GY. Organ-specific lymphatic vasculature: From development to pathophysiology. J Exp Med. 2018;215(1):35–49. https://doi.org/10.1084/jem.20171868 .
doi: 10.1084/jem.20171868 pubmed: 29242199 pmcid: 5748863
Wong BW, Zecchin A, Garcia-Caballero M, Carmeliet P. Emerging Concepts in Organ-Specific Lymphatic Vessels and Metabolic Regulation of Lymphatic Development. Dev Cell. 2018;45(3):289–301. https://doi.org/10.1016/j.devcel.2018.03.021 .
doi: 10.1016/j.devcel.2018.03.021 pubmed: 29738709
Maldonado-Zimbron VE, Hong J, Russell P, Trevaskis NL, Windsor JA, Phillips ARJ. Methods for studying pulmonary lymphatics. Eur Respir J. 2021;57(5). https://doi.org/10.1183/13993003.04106-2020 .
Chavhan GB, Amaral JG, Temple M, Itkin M. MR Lymphangiography in Children: Technique and Potential Applications. Radiographics. 2017;37(6):1775–90. https://doi.org/10.1148/rg.2017170014 .
doi: 10.1148/rg.2017170014 pubmed: 29019760
Schraufnagel DE. Lung lymphatic anatomy and correlates. Pathophysiology. 2010;17(4):337–43. https://doi.org/10.1016/j.pathophys.2009.10.008 .
doi: 10.1016/j.pathophys.2009.10.008 pubmed: 20004086
El-Chemaly S, Levine SJ, Moss J. Lymphatics in lung disease. Ann N Y Acad Sci. 2008;1131:195–202. https://doi.org/10.1196/annals.1413.017 .
doi: 10.1196/annals.1413.017 pubmed: 18519971 pmcid: 2946892
Stump B, Cui Y, Kidambi P, Lamattina AM, El-Chemaly S. Lymphatic Changes in Respiratory Diseases: More than Just Remodeling of the Lung? Am J Respir Cell Mol Biol. 2017;57(3):272–9. https://doi.org/10.1165/rcmb.2016-0290TR .
doi: 10.1165/rcmb.2016-0290TR pubmed: 28443685 pmcid: 5625224
Kulkarni RM, Herman A, Ikegami M, Greenberg JM, Akeson AL. Lymphatic ontogeny and effect of hypoplasia in developing lung. Mech Dev. 2011;128(1–2):29–40. https://doi.org/10.1016/j.mod.2010.09.003 .
doi: 10.1016/j.mod.2010.09.003 pubmed: 20932899
Itkin M, Chidekel A, Ryan KA, Rabinowitz D. Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment. Paediatr Respir Rev. 2020;36:15–24. https://doi.org/10.1016/j.prrv.2020.07.001 .
doi: 10.1016/j.prrv.2020.07.001 pubmed: 32792289
Szotak-Ajtay K, Szoke D, Kovacs G, Andreka J, Brenner GB, Giricz Z, et al. Reduced Prenatal Pulmonary Lymphatic Function Is Observed in Clp1 (K/K) Embryos With Impaired Motor Functions Including Fetal Breathing Movements in Preparation of the Developing Lung for Inflation at Birth. Front Bioeng Biotechnol. 2020;8:136. https://doi.org/10.3389/fbioe.2020.00136 .
doi: 10.3389/fbioe.2020.00136 pubmed: 32211389 pmcid: 7067749
Jakus Z, Gleghorn JP, Enis DR, Sen A, Chia S, Liu X, et al. Lymphatic function is required prenatally for lung inflation at birth. J Exp Med. 2014;211(5):815–26. https://doi.org/10.1084/jem.20132308 .
doi: 10.1084/jem.20132308 pubmed: 24733830 pmcid: 4010903
Reed HO, Wang L, Sonett J, Chen M, Yang J, Li L, et al. Lymphatic impairment leads to pulmonary tertiary lymphoid organ formation and alveolar damage. J Clin Invest. 2019;129(6):2514–26. https://doi.org/10.1172/JCI125044 .
doi: 10.1172/JCI125044 pubmed: 30946031 pmcid: 6546450
Choi HS, Ashitate Y, Lee JH, Kim SH, Matsui A, Insin N, et al. Rapid translocation of nanoparticles from the lung airspaces to the body. Nat Biotechnol. 2010;28(12):1300–3. https://doi.org/10.1038/nbt.1696 .
doi: 10.1038/nbt.1696 pubmed: 21057497 pmcid: 3058321
Solari E, Marcozzi C, Ottaviani C, Negrini D, Moriondo A. Draining the Pleural Space: Lymphatic Vessels Facing the Most Challenging Task. Biology (Basel). 2022;11(3):419. https://doi.org/10.3390/biology11030419 .
doi: 10.3390/biology11030419 pubmed: 35336793 pmcid: 8945018
Riley LE, Ataya A. Clinical approach and review of causes of a chylothorax. Respir Med. 2019;157:7–13. https://doi.org/10.1016/j.rmed.2019.08.014 .
doi: 10.1016/j.rmed.2019.08.014 pubmed: 31454675
Pieper CC, Wagenpfeil J, Henkel A, Geiger S, Koster T, Hoss K, et al. MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome. Sci Rep. 2022;12(1):11164. https://doi.org/10.1038/s41598-022-13806-w .
doi: 10.1038/s41598-022-13806-w pubmed: 35778409 pmcid: 9249771
Downie L, Sasi A, Malhotra A. Congenital chylothorax: associations and neonatal outcomes. J Paediatr Child Health. 2014;50(3):234–8. https://doi.org/10.1111/jpc.12477 .
doi: 10.1111/jpc.12477 pubmed: 24372911
Janardhan HP, Jung R, Trivedi CM. Lymphatic System in Organ Development, Function, and Regeneration. Circ Res. 2023;132(9):1181–4. https://doi.org/10.1161/CIRCRESAHA.123.322867 .
doi: 10.1161/CIRCRESAHA.123.322867 pubmed: 37104565 pmcid: 10155258
Janardhan HP, Dresser K, Hutchinson L, Trivedi CM. Pathological MAPK activation-mediated lymphatic basement membrane disruption causes lymphangiectasia that is treatable with ravoxertinib. JCI Insight. 2022;7(17). https://doi.org/10.1172/jci.insight.153033 .
Ohtani O, Ohtani Y. Lymph circulation in the liver. Anat Rec (Hoboken). 2008;291(6):643–52. https://doi.org/10.1002/ar.20681 .
doi: 10.1002/ar.20681 pubmed: 18484610
Tanaka M, Iwakiri Y. The Hepatic Lymphatic Vascular System: Structure, Function, Markers, and Lymphangiogenesis. Cell Mol Gastroenterol Hepatol. 2016;2(6):733–49. https://doi.org/10.1016/j.jcmgh.2016.09.002 .
doi: 10.1016/j.jcmgh.2016.09.002 pubmed: 28105461 pmcid: 5240041
Frenkel NC, Poghosyan S, Verheem A, Padera TP, Rinkes I, Kranenburg O, et al. Liver lymphatic drainage patterns follow segmental anatomy in a murine model. Sci Rep. 2020;10(1):21808. https://doi.org/10.1038/s41598-020-78727-y .
doi: 10.1038/s41598-020-78727-y pubmed: 33311587 pmcid: 7732834
Smith CL, Liu M, Saravanan M, Dewitt AG, Biko DM, Pinto EM, et al. Liver lymphatic anatomy and role in systemic lymphatic disease. Eur Radiol. 2022;32(1):112–21. https://doi.org/10.1007/s00330-021-08098-z .
doi: 10.1007/s00330-021-08098-z pubmed: 34165621
Halpern KB, Shenhav R, Massalha H, Toth B, Egozi A, Massasa EE, et al. Paired-cell sequencing enables spatial gene expression mapping of liver endothelial cells. Nat Biotechnol. 2018;36(10):962–70. https://doi.org/10.1038/nbt.4231 .
doi: 10.1038/nbt.4231 pubmed: 30222169 pmcid: 6546596
Inverso D, Shi J, Lee KH, Jakab M, Ben-Moshe S, Kulkarni SR, et al. A spatial vascular transcriptomic, proteomic, and phosphoproteomic atlas unveils an angiocrine Tie-Wnt signaling axis in the liver. Dev Cell. 2021;56(11):1677-93 e10. https://doi.org/10.1016/j.devcel.2021.05.001 .
doi: 10.1016/j.devcel.2021.05.001 pubmed: 34038707 pmcid: 8191494
Aziz H, Brown ZJ, Baghdadi A, Kamel IR, Pawlik TM. A Comprehensive Review of Hepatic Hemangioma Management. J Gastrointest Surg. 2022;26(9):1998–2007. https://doi.org/10.1007/s11605-022-05382-1 .
doi: 10.1007/s11605-022-05382-1 pubmed: 35705835
Janardhan HP, Meng X, Dresser K, Hutchinson L, Trivedi CM. KRAS or BRAF mutations cause hepatic vascular cavernomas treatable with MAP2K-MAPK1 inhibition. J Exp Med. 2020;217(7):e20192205. https://doi.org/10.1084/jem.20192205 .
doi: 10.1084/jem.20192205 pubmed: 32405640 pmcid: 7336315
Ugwu N, Atzmony L, Ellis KT, Panse G, Jain D, Ko CJ, et al. Cutaneous and hepatic vascular lesions due to a recurrent somatic GJA4 mutation reveal a pathway for vascular malformation. HGG Adv. 2021;2(2). https://doi.org/10.1016/j.xhgg.2021.100028 . https://doi.org/10.1016/j.xhgg.2021.100028 . Epub 2021 Mar 1.
Chung C, Iwakiri Y. The lymphatic vascular system in liver diseases: its role in ascites formation. Clin Mol Hepatol. 2013;19(2):99–104. https://doi.org/10.3350/cmh.2013.19.2.99 .
doi: 10.3350/cmh.2013.19.2.99 pubmed: 23837133 pmcid: 3701854
Tamburini BAJ, Finlon JM, Gillen AE, Kriss MS, Riemondy KA, Fu R, et al. Chronic Liver Disease in Humans Causes Expansion and Differentiation of Liver Lymphatic Endothelial Cells. Front Immunol. 2019;10:1036. https://doi.org/10.3389/fimmu.2019.01036 .
doi: 10.3389/fimmu.2019.01036 pubmed: 31156626 pmcid: 6530422
Cui Y, Liu K, Monzon-Medina ME, Padera RF, Wang H, George G, et al. Therapeutic lymphangiogenesis ameliorates established acute lung allograft rejection. J Clin Invest. 2015;125(11):4255–68. https://doi.org/10.1172/JCI79693 .
doi: 10.1172/JCI79693 pubmed: 26485284 pmcid: 4639995
Maruyama K, Miyagawa-Tomita S, Mizukami K, Matsuzaki F, Kurihara H. Isl1-expressing non-venous cell lineage contributes to cardiac lymphatic vessel development. Dev Biol. 2019;452(2):134–43. https://doi.org/10.1016/j.ydbio.2019.05.002 .
doi: 10.1016/j.ydbio.2019.05.002 pubmed: 31112709
Lioux G, Liu X, Temino S, Oxendine M, Ayala E, Ortega S, et al. A Second Heart Field-Derived Vasculogenic Niche Contributes to Cardiac Lymphatics. Dev Cell. 2020;52(3):350-63 e6. https://doi.org/10.1016/j.devcel.2019.12.006 .
doi: 10.1016/j.devcel.2019.12.006 pubmed: 31928974 pmcid: 7374559
Klotz L, Norman S, Vieira JM, Masters M, Rohling M, Dube KN, et al. Cardiac lymphatics are heterogeneous in origin and respond to injury. Nature. 2015;522(7554):62–7. https://doi.org/10.1038/nature14483 .
doi: 10.1038/nature14483 pubmed: 25992544 pmcid: 4458138
Liu X, De la Cruz E, Gu X, Balint L, Oxendine-Burns M, Terrones T, et al. Lymphoangiocrine signals promote cardiac growth and repair. Nature. 2020;588(7839):705–11. https://doi.org/10.1038/s41586-020-2998-x .
doi: 10.1038/s41586-020-2998-x pubmed: 33299187 pmcid: 7770123
Gancz D, Raftrey BC, Perlmoter G, Marin-Juez R, Semo J, Matsuoka RL, et al. Distinct origins and molecular mechanisms contribute to lymphatic formation during cardiac growth and regeneration. Elife. 2019;8:e44153. https://doi.org/10.7554/eLife.44153 .
doi: 10.7554/eLife.44153 pubmed: 31702554 pmcid: 6881115
Monaghan RM, Naylor RW, Flatman D, Kasher PR, Williams SG, Keavney BD. FLT4 causes developmental disorders of the cardiovascular and lymphovascular systems via pleiotropic molecular mechanisms. Cardiovasc Res. 2024. https://doi.org/10.1093/cvr/cvae104 .
doi: 10.1093/cvr/cvae104 pubmed: 38713105
Maruyama K, Naemura K, Arima Y, Uchijima Y, Nagao H, Yoshihara K, et al. Semaphorin3E-PlexinD1 signaling in coronary artery and lymphatic vessel development with clinical implications in myocardial recovery. iScience. 2021;24(4):102305. https://doi.org/10.1016/j.isci.2021.102305 .
doi: 10.1016/j.isci.2021.102305 pubmed: 33870127 pmcid: 8041864
Heron C, Dumesnil A, Houssari M, Renet S, Lemarcis T, Lebon A, et al. Regulation and impact of cardiac lymphangiogenesis in pressure-overload-induced heart failure. Cardiovasc Res. 2023;119(2):492–505. https://doi.org/10.1093/cvr/cvac086 .
doi: 10.1093/cvr/cvac086 pubmed: 35689481
Cooper STE, Lokman AB, Riley PR. Role of the Lymphatics in Cardiac Disease. Arterioscler Thromb Vasc Biol. 2024;44(6):1181–90. https://doi.org/10.1161/ATVBAHA.124.319854 .
doi: 10.1161/ATVBAHA.124.319854 pubmed: 38634279
Henri O, Pouehe C, Houssari M, Galas L, Nicol L, Edwards-Levy F, et al. Selective Stimulation of Cardiac Lymphangiogenesis Reduces Myocardial Edema and Fibrosis Leading to Improved Cardiac Function Following Myocardial Infarction. Circulation. 2016;133(15):1484–97. https://doi.org/10.1161/CIRCULATIONAHA.115.020143 .
doi: 10.1161/CIRCULATIONAHA.115.020143 pubmed: 26933083
Keller TCSt, Lim L, Shewale SV, McDaid K, Marti-Pamies I, Tang AT, et al. Genetic blockade of lymphangiogenesis does not impair cardiac function after myocardial infarction. J Clin Invest. 2021;131(20). https://doi.org/10.1172/JCI147070 .

Auteurs

Harish P Janardhan (HP)

Division of Cardiovascular Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA.
Department of Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA.

Brianna T Wachter (BT)

Division of Cardiovascular Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA.
Department of Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA.
MD-PhD Program, Morningside Graduate School of Biomedical Sciences, UMass Chan Medical School, Worcester, MA, 01605, USA.

Chinmay M Trivedi (CM)

Division of Cardiovascular Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA. chinmay.trivedi@umassmed.edu.
Department of Medicine, UMass Chan Medical School, Worcester, MA, 01605, USA. chinmay.trivedi@umassmed.edu.
MD-PhD Program, Morningside Graduate School of Biomedical Sciences, UMass Chan Medical School, Worcester, MA, 01605, USA. chinmay.trivedi@umassmed.edu.
Department of Molecular, Cell, and Cancer Biology, UMass Chan Medical School, Worcester, MA, 01605, USA. chinmay.trivedi@umassmed.edu.

Classifications MeSH