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Type: Journal article
Title: Pkd1 regulates lymphatic vascular morphogenesis during development
Author: Coxam, B.
Sabine, A.
Bower, N.
Smith, K.
Pichol-Thievend, C.
Skoczylas, R.
Astin, J.
Frampton, E.
Jaquet, M.
Crosier, P.
Parton, R.
Harvey, N.
Petrova, T.
Schulte-Merker, S.
Francois, M.
Hogan, B.
Citation: Cell Reports, 2014; 7(3):623-633
Publisher: Elsevier (Cell Press)
Issue Date: 2014
ISSN: 2211-1247
Statement of
Baptiste Coxam, Amélie Sabine, Neil I. Bower, Kelly A. Smith, Cathy Pichol-Thievend, Renae Skoczylas, Jonathan W. Astin, Emmanuelle Frampton, Muriel Jaquet, Philip S. Crosier, Robert G. Parton, Natasha L. Harvey, Tatiana V. Petrova, Stefan Schulte-Merker, Mathias Francois, Benjamin M. Hogan
Abstract: Lymphatic vessels arise during development through sprouting of precursor cells from veins, which is regulated by known signaling and transcriptional mechanisms. The ongoing elaboration of vessels to form a network is less well understood. This involves cell polarization, coordinated migration, adhesion, mixing, regression, and shape rearrangements. We identified a zebrafish mutant, lymphatic and cardiac defects 1 (lyc1), with reduced lymphatic vessel development. A mutation in polycystic kidney disease 1a was responsible for the phenotype. PKD1 is the most frequently mutated gene in autosomal dominant polycystic kidney disease (ADPKD). Initial lymphatic precursor sprouting is normal in lyc1 mutants, but ongoing migration fails. Loss of Pkd1 in mice has no effect on precursor sprouting but leads to failed morphogenesis of the subcutaneous lymphatic network. Individual lymphatic endothelial cells display defective polarity, elongation, and adherens junctions. This work identifies a highly selective and unexpected role for Pkd1 in lymphatic vessel morphogenesis during development.
Keywords: Lymphatic Vessels
Mice, Inbred C57BL
Animals, Genetically Modified
Lymph Nodes
Endothelial Cells
Cells, Cultured
Intercellular Junctions
Polycystic Kidney, Autosomal Dominant
Zebrafish Proteins
Embryonic Development
TRPP Cation Channels
Embryo, Mammalian
Mice, Transgenic
Rights: © 2014 The Authors. Published by Elsevier Ltd. This is an open access under the CC BY license (
DOI: 10.1016/j.celrep.2014.03.063
Grant ID:
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