Serveur d'exploration sur le lymphœdème

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Pkd1 regulates lymphatic vascular morphogenesis during development

Identifieur interne : 002693 ( Main/Exploration ); précédent : 002692; suivant : 002694

Pkd1 regulates lymphatic vascular morphogenesis during development

Auteurs : Baptiste Coxam [Australie] ; Amélie Sabine [Suisse] ; Neil I. Bower [Australie] ; Kelly A. Smith [Australie] ; Cathy Pichol-Thievend [Australie] ; Renae Skoczylas [Australie] ; Jonathan W. Astin [Nouvelle-Zélande] ; Emmanuelle Frampton [Australie] ; Muriel Jaquet [Suisse] ; Philip S. Crosier [Nouvelle-Zélande] ; Robert G. Parton [Australie] ; Natasha L. Harvey [Australie] ; Tatiana V. Petrova [Suisse] ; Stefan Schulte-Merker [Pays-Bas] ; Mathias Francois [Australie] ; Benjamin M. Hogan [Australie]

Source :

RBID : PMC:5005109

Abstract

Lymphatic vessels arise during development through sprouting of precursor cells from veins, which is regulated by well-studied signaling and transcriptional mechanisms. Less well understood is the ongoing elaboration of vessels to form a network. This involves cell polarisation, coordinated migration, adhesion, mixing, regression and cell shape rearrangements. We identified a zebrafish mutant, lymphatic and cardiac defects 1 (lyc1), with reduced lymphatic vessel development. We found a mutation in polycystic kidney disease 1a to be responsible for the phenotype. PKD1 is the most frequently mutated gene in autosomal dominant polycystic kidney disease (ADPKD). Initial sprouting of lymphatic precursors is normal in lyc1 mutants, but ongoing migration fails. Loss of Pkd1 in mice also has no effect on sprouting of precursors 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.


Url:
DOI: 10.1016/j.celrep.2014.03.063
PubMed: 24767999
PubMed Central: 5005109


Affiliations:


Links toward previous steps (curation, corpus...)


Le document en format XML

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<name sortKey="Pichol Thievend, Cathy" sort="Pichol Thievend, Cathy" uniqKey="Pichol Thievend C" first="Cathy" last="Pichol-Thievend">Cathy Pichol-Thievend</name>
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<name sortKey="Frampton, Emmanuelle" sort="Frampton, Emmanuelle" uniqKey="Frampton E" first="Emmanuelle" last="Frampton">Emmanuelle Frampton</name>
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<p id="P1">Lymphatic vessels arise during development through sprouting of precursor cells from veins, which is regulated by well-studied signaling and transcriptional mechanisms. Less well understood is the ongoing elaboration of vessels to form a network. This involves cell polarisation, coordinated migration, adhesion, mixing, regression and cell shape rearrangements. We identified a zebrafish mutant,
<italic>lymphatic and cardiac defects 1 (lyc1),</italic>
with reduced lymphatic vessel development. We found a mutation in
<italic>polycystic kidney disease 1a</italic>
to be responsible for the phenotype.
<italic>PKD1</italic>
is the most frequently mutated gene in autosomal dominant polycystic kidney disease (ADPKD). Initial sprouting of lymphatic precursors is normal in
<italic>lyc1</italic>
mutants, but ongoing migration fails. Loss of
<italic>Pkd1</italic>
in mice also has no effect on sprouting of precursors 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
<italic>Pkd1</italic>
in lymphatic vessel morphogenesis during development.</p>
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<name sortKey="Francois, Mathias" sort="Francois, Mathias" uniqKey="Francois M" first="Mathias" last="Francois">Mathias Francois</name>
<name sortKey="Harvey, Natasha L" sort="Harvey, Natasha L" uniqKey="Harvey N" first="Natasha L." last="Harvey">Natasha L. Harvey</name>
<name sortKey="Hogan, Benjamin M" sort="Hogan, Benjamin M" uniqKey="Hogan B" first="Benjamin M." last="Hogan">Benjamin M. Hogan</name>
<name sortKey="Parton, Robert G" sort="Parton, Robert G" uniqKey="Parton R" first="Robert G." last="Parton">Robert G. Parton</name>
<name sortKey="Pichol Thievend, Cathy" sort="Pichol Thievend, Cathy" uniqKey="Pichol Thievend C" first="Cathy" last="Pichol-Thievend">Cathy Pichol-Thievend</name>
<name sortKey="Skoczylas, Renae" sort="Skoczylas, Renae" uniqKey="Skoczylas R" first="Renae" last="Skoczylas">Renae Skoczylas</name>
<name sortKey="Smith, Kelly A" sort="Smith, Kelly A" uniqKey="Smith K" first="Kelly A." last="Smith">Kelly A. Smith</name>
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<name sortKey="Sabine, Amelie" sort="Sabine, Amelie" uniqKey="Sabine A" first="Amélie" last="Sabine">Amélie Sabine</name>
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<name sortKey="Jaquet, Muriel" sort="Jaquet, Muriel" uniqKey="Jaquet M" first="Muriel" last="Jaquet">Muriel Jaquet</name>
<name sortKey="Petrova, Tatiana V" sort="Petrova, Tatiana V" uniqKey="Petrova T" first="Tatiana V." last="Petrova">Tatiana V. Petrova</name>
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<country name="Nouvelle-Zélande">
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<name sortKey="Astin, Jonathan W" sort="Astin, Jonathan W" uniqKey="Astin J" first="Jonathan W." last="Astin">Jonathan W. Astin</name>
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<name sortKey="Crosier, Philip S" sort="Crosier, Philip S" uniqKey="Crosier P" first="Philip S." last="Crosier">Philip S. Crosier</name>
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<name sortKey="Schulte Merker, Stefan" sort="Schulte Merker, Stefan" uniqKey="Schulte Merker S" first="Stefan" last="Schulte-Merker">Stefan Schulte-Merker</name>
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</record>

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