Defective valves and abnormal mural cell recruitment underlie lymphatic vascular failure in lymphedema distichiasis.
Identifieur interne : 008829 ( Main/Exploration ); précédent : 008828; suivant : 008830Defective valves and abnormal mural cell recruitment underlie lymphatic vascular failure in lymphedema distichiasis.
Auteurs : Tatiana V. Petrova [Finlande] ; Terhi Karpanen ; Camilla Norrmén ; Russell Mellor ; Tomoki Tamakoshi ; David Finegold ; Robert Ferrell ; Dontscho Kerjaschki ; Peter Mortimer (dermatologue) [Royaume-Uni] ; Seppo Yl Herttuala ; Naoyuki Miura ; Kari Alitalo [Finlande]Source :
- Nature medicine [ 1078-8956 ] ; 2004.
Descripteurs français
- KwdFr :
- ARN (génétique), Animaux, Bleu d'Evans, Cellules cultivées, Facteurs de transcription (génétique), Facteurs de transcription (métabolisme), Facteurs de transcription Forkhead, Humains, Hybridation in situ, Immunohistochimie, Lymphangiogenèse (génétique), Lymphoedème (anatomopathologie), Lymphoedème (génétique), Malformations lymphatiques (anatomopathologie), Malformations lymphatiques (génétique), Microscopie de fluorescence, Modèles animaux de maladie humaine, Mutation (génétique), Protéines de liaison à l'ADN (génétique), Protéines de liaison à l'ADN (métabolisme), Péricytes (anatomopathologie), Récepteur-3 au facteur croissance endothéliale vasculaire (métabolisme), Souches mutantes de souris, Souris, Technique de Northern, Vaisseaux lymphatiques (anatomopathologie).
- MESH :
- anatomopathologie : Lymphoedème, Malformations lymphatiques, Péricytes, Vaisseaux lymphatiques.
- génétique : ARN, Facteurs de transcription, Lymphangiogenèse, Lymphoedème, Malformations lymphatiques, Mutation, Protéines de liaison à l'ADN.
- métabolisme : Facteurs de transcription, Protéines de liaison à l'ADN, Récepteur-3 au facteur croissance endothéliale vasculaire.
- Animaux, Bleu d'Evans, Cellules cultivées, Facteurs de transcription Forkhead, Humains, Hybridation in situ, Immunohistochimie, Microscopie de fluorescence, Modèles animaux de maladie humaine, Souches mutantes de souris, Souris, Technique de Northern.
English descriptors
- KwdEn :
- Animals, Blotting, Northern, Cells, Cultured, DNA-Binding Proteins (genetics), DNA-Binding Proteins (metabolism), Disease Models, Animal, Evans Blue, Forkhead Transcription Factors, Humans, Immunohistochemistry, In Situ Hybridization, Lymphangiogenesis (genetics), Lymphatic Abnormalities (genetics), Lymphatic Abnormalities (pathology), Lymphatic Vessels (pathology), Lymphedema (genetics), Lymphedema (pathology), Mice, Mice, Mutant Strains, Microscopy, Fluorescence, Mutation (genetics), Pericytes (pathology), RNA (genetics), Transcription Factors (genetics), Transcription Factors (metabolism), Vascular Endothelial Growth Factor Receptor-3 (metabolism).
- MESH :
- chemical , genetics : DNA-Binding Proteins, RNA, Transcription Factors.
- chemical , metabolism : DNA-Binding Proteins, Transcription Factors, Vascular Endothelial Growth Factor Receptor-3.
- genetics : Lymphangiogenesis, Lymphatic Abnormalities, Lymphedema, Mutation.
- pathology : Lymphatic Abnormalities, Lymphatic Vessels, Lymphedema, Pericytes.
- Animals, Blotting, Northern, Cells, Cultured, Disease Models, Animal, Evans Blue, Forkhead Transcription Factors, Humans, Immunohistochemistry, In Situ Hybridization, Mice, Mice, Mutant Strains, Microscopy, Fluorescence.
Abstract
Lymphatic vessels are essential for the removal of interstitial fluid and prevention of tissue edema. Lymphatic capillaries lack associated mural cells, and collecting lymphatic vessels have valves, which prevent lymph backflow. In lymphedema-distichiasis (LD), lymphatic vessel function fails because of mutations affecting the forkhead transcription factor FOXC2. We report that Foxc2(-/-) mice show abnormal lymphatic vascular patterning, increased pericyte investment of lymphatic vessels, agenesis of valves and lymphatic dysfunction. In addition, an abnormally large proportion of skin lymphatic vessels was covered with smooth muscle cells in individuals with LD and in mice heterozygous for Foxc2 and for the gene encoding lymphatic endothelial receptor, Vegfr3 (also known as Flt4). Our data show that Foxc2 is essential for the morphogenesis of lymphatic valves and the establishment of a pericyte-free lymphatic capillary network and that it cooperates with Vegfr3 in the latter process. Our results indicate that an abnormal interaction between the lymphatic endothelial cells and pericytes, as well as valve defects, underlie the pathogenesis of LD.
DOI: 10.1038/nm1094
PubMed: 15322537
Affiliations:
- Finlande, Royaume-Uni
- Angleterre, Grand Londres, Uusimaa
- Helsinki, Londres
- Université d'Helsinki, Université de Londres
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Le document en format XML
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Petrova</name><affiliation wicri:level="1"><nlm:affiliation>Molecular/Cancer Biology Laboratory and Ludwig Institute for Cancer Research, Biomedicum Helsinki and Helsinki University Central Hospital, University of Helsinki, Haartmaninkatu 8, P.O.B. 63, 00014 Helsinki, Finland.</nlm:affiliation><country xml:lang="fr">Finlande</country><wicri:regionArea>Molecular/Cancer Biology Laboratory and Ludwig Institute for Cancer Research, Biomedicum Helsinki and Helsinki University Central Hospital, University of Helsinki, Haartmaninkatu 8, P.O.B. 63, 00014 Helsinki</wicri:regionArea><wicri:noRegion>00014 Helsinki</wicri:noRegion></affiliation></author><author><name sortKey="Karpanen, Terhi" sort="Karpanen, Terhi" uniqKey="Karpanen T" first="Terhi" last="Karpanen">Terhi Karpanen</name></author><author><name sortKey="Norrmen, Camilla" sort="Norrmen, Camilla" uniqKey="Norrmen C" first="Camilla" last="Norrmén">Camilla Norrmén</name></author><author><name sortKey="Mellor, Russell" sort="Mellor, 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Factors (metabolism)</term><term>Vascular Endothelial Growth Factor Receptor-3 (metabolism)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>ARN (génétique)</term><term>Animaux</term><term>Bleu d'Evans</term><term>Cellules cultivées</term><term>Facteurs de transcription (génétique)</term><term>Facteurs de transcription (métabolisme)</term><term>Facteurs de transcription Forkhead</term><term>Humains</term><term>Hybridation in situ</term><term>Immunohistochimie</term><term>Lymphangiogenèse (génétique)</term><term>Lymphoedème (anatomopathologie)</term><term>Lymphoedème (génétique)</term><term>Malformations lymphatiques (anatomopathologie)</term><term>Malformations lymphatiques (génétique)</term><term>Microscopie de fluorescence</term><term>Modèles animaux de maladie humaine</term><term>Mutation (génétique)</term><term>Protéines de liaison à l'ADN (génétique)</term><term>Protéines de liaison à l'ADN (métabolisme)</term><term>Péricytes (anatomopathologie)</term><term>Récepteur-3 au facteur croissance endothéliale vasculaire (métabolisme)</term><term>Souches mutantes de souris</term><term>Souris</term><term>Technique de Northern</term><term>Vaisseaux lymphatiques (anatomopathologie)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>DNA-Binding Proteins</term><term>RNA</term><term>Transcription Factors</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>DNA-Binding Proteins</term><term>Transcription Factors</term><term>Vascular Endothelial Growth Factor Receptor-3</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Lymphoedème</term><term>Malformations lymphatiques</term><term>Péricytes</term><term>Vaisseaux lymphatiques</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Lymphangiogenesis</term><term>Lymphatic 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Factors</term><term>Humans</term><term>Immunohistochemistry</term><term>In Situ Hybridization</term><term>Mice</term><term>Mice, Mutant Strains</term><term>Microscopy, Fluorescence</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Animaux</term><term>Bleu d'Evans</term><term>Cellules cultivées</term><term>Facteurs de transcription Forkhead</term><term>Humains</term><term>Hybridation in situ</term><term>Immunohistochimie</term><term>Microscopie de fluorescence</term><term>Modèles animaux de maladie humaine</term><term>Souches mutantes de souris</term><term>Souris</term><term>Technique de Northern</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Lymphatic vessels are essential for the removal of interstitial fluid and prevention of tissue edema. Lymphatic capillaries lack associated mural cells, and collecting lymphatic vessels have valves, which prevent lymph backflow. In lymphedema-distichiasis (LD), lymphatic vessel function fails because of mutations affecting the forkhead transcription factor FOXC2. We report that Foxc2(-/-) mice show abnormal lymphatic vascular patterning, increased pericyte investment of lymphatic vessels, agenesis of valves and lymphatic dysfunction. In addition, an abnormally large proportion of skin lymphatic vessels was covered with smooth muscle cells in individuals with LD and in mice heterozygous for Foxc2 and for the gene encoding lymphatic endothelial receptor, Vegfr3 (also known as Flt4). Our data show that Foxc2 is essential for the morphogenesis of lymphatic valves and the establishment of a pericyte-free lymphatic capillary network and that it cooperates with Vegfr3 in the latter process. Our results indicate that an abnormal interaction between the lymphatic endothelial cells and pericytes, as well as valve defects, underlie the pathogenesis of LD.</div></front></TEI><affiliations><list><country><li>Finlande</li><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li><li>Uusimaa</li></region><settlement><li>Helsinki</li><li>Londres</li></settlement><orgName><li>Université d'Helsinki</li><li>Université de Londres</li></orgName></list><tree><noCountry><name sortKey="Ferrell, Robert" sort="Ferrell, Robert" uniqKey="Ferrell R" first="Robert" last="Ferrell">Robert Ferrell</name><name sortKey="Finegold, David" sort="Finegold, David" uniqKey="Finegold D" first="David" last="Finegold">David Finegold</name><name sortKey="Karpanen, Terhi" sort="Karpanen, Terhi" uniqKey="Karpanen T" first="Terhi" last="Karpanen">Terhi Karpanen</name><name sortKey="Kerjaschki, Dontscho" sort="Kerjaschki, Dontscho" uniqKey="Kerjaschki D" first="Dontscho" last="Kerjaschki">Dontscho Kerjaschki</name><name sortKey="Mellor, Russell" sort="Mellor, Russell" uniqKey="Mellor R" first="Russell" last="Mellor">Russell Mellor</name><name sortKey="Miura, Naoyuki" sort="Miura, Naoyuki" uniqKey="Miura N" first="Naoyuki" last="Miura">Naoyuki Miura</name><name sortKey="Norrmen, Camilla" sort="Norrmen, Camilla" uniqKey="Norrmen C" first="Camilla" last="Norrmén">Camilla Norrmén</name><name sortKey="Tamakoshi, Tomoki" sort="Tamakoshi, Tomoki" uniqKey="Tamakoshi T" first="Tomoki" last="Tamakoshi">Tomoki Tamakoshi</name><name sortKey="Yl Herttuala, Seppo" sort="Yl Herttuala, Seppo" uniqKey="Yl Herttuala S" first="Seppo" last="Yl Herttuala">Seppo Yl Herttuala</name></noCountry><country name="Finlande"><noRegion><name sortKey="Petrova, Tatiana V" sort="Petrova, Tatiana V" uniqKey="Petrova T" first="Tatiana V" last="Petrova">Tatiana V. Petrova</name></noRegion><name sortKey="Alitalo, Kari" sort="Alitalo, Kari" uniqKey="Alitalo K" first="Kari" last="Alitalo">Kari Alitalo</name></country><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Mortimer, Peter" sort="Mortimer, Peter" uniqKey="Mortimer P" first="Peter" last="Mortimer">Peter Mortimer (dermatologue)</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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