Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/90542
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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
2211-1247
Statement of
Responsibility: 
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; Lymphangiogenesis; Phenotype; Mutation; 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 (http://creativecommons.org/licenses/by/3.0/).
RMID: 0030026592
DOI: 10.1016/j.celrep.2014.03.063
Grant ID: http://purl.org/au-research/grants/arc/FT100100165
http://purl.org/au-research/grants/arc/FT110100496
http://purl.org/au-research/grants/nhmrc/1011242
http://purl.org/au-research/grants/nhmrc/569542
http://purl.org/au-research/grants/nhmrc/631657
Appears in Collections:Medicine publications

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