Sox18 and Sox7 play redundant roles in vascular development.
Identifieur interne : 006935 ( Main/Exploration ); précédent : 006934; suivant : 006936Sox18 and Sox7 play redundant roles in vascular development.
Auteurs : Solei Cermenati [Italie] ; Silvia Moleri ; Simona Cimbro ; Paola Corti ; Luca Del Giacco ; Roberta Amodeo ; Elisabetta Dejana ; Peter Koopman ; Franco Cotelli ; Monica BeltrameSource :
- Blood [ 0006-4971 ] ; 2008.
Descripteurs français
- KwdFr :
- Animal génétiquement modifié, Animaux, Cellules endothéliales (métabolisme), Circulation sanguine, Danio zébré (embryologie), Danio zébré (génétique), Embryon non mammalien (malformations), Facteurs de transcription SOX-F, Gènes rapporteurs, Marqueurs biologiques (métabolisme), Mutation (génétique), Protéines de liaison à l'ADN (génétique), Protéines de liaison à l'ADN (métabolisme), Protéines de poisson-zèbre (génétique), Protéines de poisson-zèbre (métabolisme), Régulation de l'expression des gènes au cours du développement, Similitude de séquences d'acides aminés, Spécificité d'organe, Vaisseaux sanguins (embryologie), Vaisseaux sanguins (malformations).
- MESH :
- embryologie : Danio zébré, Vaisseaux sanguins.
- génétique : Danio zébré, Mutation, Protéines de liaison à l'ADN, Protéines de poisson-zèbre.
- malformations : Embryon non mammalien, Vaisseaux sanguins.
- métabolisme : Cellules endothéliales, Marqueurs biologiques, Protéines de liaison à l'ADN, Protéines de poisson-zèbre.
- Animal génétiquement modifié, Animaux, Circulation sanguine, Facteurs de transcription SOX-F, Gènes rapporteurs, Régulation de l'expression des gènes au cours du développement, Similitude de séquences d'acides aminés, Spécificité d'organe.
English descriptors
- KwdEn :
- Animals, Animals, Genetically Modified, Biomarkers (metabolism), Blood Circulation, Blood Vessels (abnormalities), Blood Vessels (embryology), DNA-Binding Proteins (genetics), DNA-Binding Proteins (metabolism), Embryo, Nonmammalian (abnormalities), Endothelial Cells (metabolism), Gene Expression Regulation, Developmental, Genes, Reporter, Mutation (genetics), Organ Specificity, SOXF Transcription Factors, Sequence Homology, Amino Acid, Zebrafish (embryology), Zebrafish (genetics), Zebrafish Proteins (genetics), Zebrafish Proteins (metabolism).
- MESH :
- chemical , genetics : DNA-Binding Proteins, Zebrafish Proteins.
- chemical , metabolism : Biomarkers, DNA-Binding Proteins, Zebrafish Proteins.
- abnormalities : Blood Vessels, Embryo, Nonmammalian.
- embryology : Blood Vessels, Zebrafish.
- genetics : Mutation, Zebrafish.
- metabolism : Endothelial Cells.
- Animals, Animals, Genetically Modified, Blood Circulation, Gene Expression Regulation, Developmental, Genes, Reporter, Organ Specificity, SOXF Transcription Factors, Sequence Homology, Amino Acid.
Abstract
Mutations in SOX18 cause the human hypotrichosis-lymphedema-telangiectasia (HLT) syndrome. Their murine counterparts are the spontaneous ragged mutants, showing combined defects in hair follicle, blood vessel, and lymphatic vessel development. Mice null for Sox18 display only mild coat defects, suggesting a dominant-negative effect of Sox18/ragged mutations and functional redundancy between Sox18 and other Sox-F proteins. We addressed this point in zebrafish. The zebrafish homologs of Sox18 and of Sox7 are expressed in angioblasts and in the endothelial component of nascent blood vessels in embryos. Knockdown of either gene, using moderate doses of specific morpholinos, had minimal effects on vessels. In contrast, simultaneous knockdown of both genes resulted in multiple fusions between the major axial vessels. With combined use of transgenic lines and molecular markers, we could show that endothelial cells are specified, but fail to acquire a correct arteriovenous identity. Venous endothelial cell differentiation was more severely affected than arterial. Thus, sox7 and sox18 play redundant but collectively essential roles in the establishment of proper arteriovenous identity in zebrafish. Our data suggest that a defect in arteriovenous identity could be responsible for the formation of telangiectases in patients with HLT.
DOI: 10.1182/blood-2007-07-100412
PubMed: 18094332
Affiliations:
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last="Cimbro">Simona Cimbro</name></author><author><name sortKey="Corti, Paola" sort="Corti, Paola" uniqKey="Corti P" first="Paola" last="Corti">Paola Corti</name></author><author><name sortKey="Del Giacco, Luca" sort="Del Giacco, Luca" uniqKey="Del Giacco L" first="Luca" last="Del Giacco">Luca Del Giacco</name></author><author><name sortKey="Amodeo, Roberta" sort="Amodeo, Roberta" uniqKey="Amodeo R" first="Roberta" last="Amodeo">Roberta Amodeo</name></author><author><name sortKey="Dejana, Elisabetta" sort="Dejana, Elisabetta" uniqKey="Dejana E" first="Elisabetta" last="Dejana">Elisabetta Dejana</name></author><author><name sortKey="Koopman, Peter" sort="Koopman, Peter" uniqKey="Koopman P" first="Peter" last="Koopman">Peter Koopman</name></author><author><name sortKey="Cotelli, Franco" sort="Cotelli, Franco" uniqKey="Cotelli F" first="Franco" last="Cotelli">Franco Cotelli</name></author><author><name sortKey="Beltrame, Monica" sort="Beltrame, Monica" uniqKey="Beltrame M" first="Monica" 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type="wicri:Area/Main/Curation">006935</idno><idno type="wicri:Area/Main/Exploration">006935</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Sox18 and Sox7 play redundant roles in vascular development.</title><author><name sortKey="Cermenati, Solei" sort="Cermenati, Solei" uniqKey="Cermenati S" first="Solei" last="Cermenati">Solei Cermenati</name><affiliation wicri:level="3"><nlm:affiliation>Dipartimento di Scienze Biomolecolari e Biotecnologie, Università degli Studi di Milano, Via Celoria 26, Milan, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Dipartimento di Scienze Biomolecolari e Biotecnologie, Università degli Studi di Milano, Via Celoria 26, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Moleri, Silvia" sort="Moleri, Silvia" uniqKey="Moleri S" first="Silvia" last="Moleri">Silvia Moleri</name></author><author><name sortKey="Cimbro, Simona" sort="Cimbro, Simona" uniqKey="Cimbro S" first="Simona" last="Cimbro">Simona Cimbro</name></author><author><name sortKey="Corti, Paola" sort="Corti, Paola" uniqKey="Corti P" first="Paola" last="Corti">Paola Corti</name></author><author><name sortKey="Del Giacco, Luca" sort="Del Giacco, Luca" uniqKey="Del Giacco L" first="Luca" last="Del Giacco">Luca Del Giacco</name></author><author><name sortKey="Amodeo, Roberta" sort="Amodeo, Roberta" uniqKey="Amodeo R" first="Roberta" last="Amodeo">Roberta Amodeo</name></author><author><name sortKey="Dejana, Elisabetta" sort="Dejana, Elisabetta" uniqKey="Dejana E" first="Elisabetta" last="Dejana">Elisabetta Dejana</name></author><author><name sortKey="Koopman, Peter" sort="Koopman, Peter" uniqKey="Koopman P" first="Peter" last="Koopman">Peter Koopman</name></author><author><name sortKey="Cotelli, Franco" sort="Cotelli, Franco" uniqKey="Cotelli F" first="Franco" last="Cotelli">Franco Cotelli</name></author><author><name sortKey="Beltrame, Monica" sort="Beltrame, Monica" uniqKey="Beltrame M" first="Monica" last="Beltrame">Monica Beltrame</name></author></analytic><series><title level="j">Blood</title><idno type="ISSN">0006-4971</idno><imprint><date when="2008" type="published">2008</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Animals</term><term>Animals, Genetically Modified</term><term>Biomarkers (metabolism)</term><term>Blood Circulation</term><term>Blood Vessels (abnormalities)</term><term>Blood Vessels (embryology)</term><term>DNA-Binding Proteins (genetics)</term><term>DNA-Binding Proteins (metabolism)</term><term>Embryo, Nonmammalian (abnormalities)</term><term>Endothelial Cells (metabolism)</term><term>Gene Expression Regulation, Developmental</term><term>Genes, Reporter</term><term>Mutation (genetics)</term><term>Organ Specificity</term><term>SOXF Transcription Factors</term><term>Sequence Homology, Amino Acid</term><term>Zebrafish (embryology)</term><term>Zebrafish (genetics)</term><term>Zebrafish Proteins (genetics)</term><term>Zebrafish Proteins (metabolism)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Animal génétiquement modifié</term><term>Animaux</term><term>Cellules endothéliales (métabolisme)</term><term>Circulation sanguine</term><term>Danio zébré (embryologie)</term><term>Danio zébré (génétique)</term><term>Embryon non mammalien (malformations)</term><term>Facteurs de transcription SOX-F</term><term>Gènes rapporteurs</term><term>Marqueurs biologiques (métabolisme)</term><term>Mutation (génétique)</term><term>Protéines de liaison à l'ADN (génétique)</term><term>Protéines de liaison à l'ADN (métabolisme)</term><term>Protéines de poisson-zèbre (génétique)</term><term>Protéines de poisson-zèbre (métabolisme)</term><term>Régulation de l'expression des gènes au cours du développement</term><term>Similitude de séquences d'acides aminés</term><term>Spécificité d'organe</term><term>Vaisseaux sanguins (embryologie)</term><term>Vaisseaux sanguins (malformations)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>DNA-Binding Proteins</term><term>Zebrafish Proteins</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Biomarkers</term><term>DNA-Binding Proteins</term><term>Zebrafish Proteins</term></keywords><keywords scheme="MESH" qualifier="abnormalities" xml:lang="en"><term>Blood Vessels</term><term>Embryo, Nonmammalian</term></keywords><keywords scheme="MESH" qualifier="embryologie" xml:lang="fr"><term>Danio zébré</term><term>Vaisseaux sanguins</term></keywords><keywords scheme="MESH" qualifier="embryology" xml:lang="en"><term>Blood Vessels</term><term>Zebrafish</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Mutation</term><term>Zebrafish</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Danio zébré</term><term>Mutation</term><term>Protéines de liaison à l'ADN</term><term>Protéines de poisson-zèbre</term></keywords><keywords scheme="MESH" qualifier="malformations" xml:lang="fr"><term>Embryon non mammalien</term><term>Vaisseaux sanguins</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Endothelial Cells</term></keywords><keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Cellules endothéliales</term><term>Marqueurs biologiques</term><term>Protéines de liaison à l'ADN</term><term>Protéines de poisson-zèbre</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Animals</term><term>Animals, Genetically Modified</term><term>Blood Circulation</term><term>Gene Expression Regulation, Developmental</term><term>Genes, Reporter</term><term>Organ Specificity</term><term>SOXF Transcription Factors</term><term>Sequence Homology, Amino Acid</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Animal génétiquement modifié</term><term>Animaux</term><term>Circulation sanguine</term><term>Facteurs de transcription SOX-F</term><term>Gènes rapporteurs</term><term>Régulation de l'expression des gènes au cours du développement</term><term>Similitude de séquences d'acides aminés</term><term>Spécificité d'organe</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Mutations in SOX18 cause the human hypotrichosis-lymphedema-telangiectasia (HLT) syndrome. Their murine counterparts are the spontaneous ragged mutants, showing combined defects in hair follicle, blood vessel, and lymphatic vessel development. Mice null for Sox18 display only mild coat defects, suggesting a dominant-negative effect of Sox18/ragged mutations and functional redundancy between Sox18 and other Sox-F proteins. We addressed this point in zebrafish. The zebrafish homologs of Sox18 and of Sox7 are expressed in angioblasts and in the endothelial component of nascent blood vessels in embryos. Knockdown of either gene, using moderate doses of specific morpholinos, had minimal effects on vessels. In contrast, simultaneous knockdown of both genes resulted in multiple fusions between the major axial vessels. With combined use of transgenic lines and molecular markers, we could show that endothelial cells are specified, but fail to acquire a correct arteriovenous identity. Venous endothelial cell differentiation was more severely affected than arterial. Thus, sox7 and sox18 play redundant but collectively essential roles in the establishment of proper arteriovenous identity in zebrafish. Our data suggest that a defect in arteriovenous identity could be responsible for the formation of telangiectases in patients with HLT.</div></front></TEI><affiliations><list><country><li>Italie</li></country><region><li>Lombardie</li></region><settlement><li>Milan</li></settlement></list><tree><noCountry><name sortKey="Amodeo, Roberta" sort="Amodeo, Roberta" uniqKey="Amodeo R" first="Roberta" last="Amodeo">Roberta Amodeo</name><name sortKey="Beltrame, Monica" sort="Beltrame, Monica" uniqKey="Beltrame M" first="Monica" last="Beltrame">Monica Beltrame</name><name sortKey="Cimbro, Simona" sort="Cimbro, Simona" uniqKey="Cimbro S" first="Simona" last="Cimbro">Simona Cimbro</name><name sortKey="Corti, Paola" sort="Corti, Paola" uniqKey="Corti P" first="Paola" last="Corti">Paola Corti</name><name sortKey="Cotelli, Franco" sort="Cotelli, Franco" uniqKey="Cotelli F" first="Franco" last="Cotelli">Franco Cotelli</name><name sortKey="Dejana, Elisabetta" sort="Dejana, Elisabetta" uniqKey="Dejana E" first="Elisabetta" last="Dejana">Elisabetta Dejana</name><name sortKey="Del Giacco, Luca" sort="Del Giacco, Luca" uniqKey="Del Giacco L" first="Luca" last="Del Giacco">Luca Del Giacco</name><name sortKey="Koopman, Peter" sort="Koopman, Peter" uniqKey="Koopman P" first="Peter" last="Koopman">Peter Koopman</name><name sortKey="Moleri, Silvia" sort="Moleri, Silvia" uniqKey="Moleri S" first="Silvia" last="Moleri">Silvia Moleri</name></noCountry><country name="Italie"><region name="Lombardie"><name sortKey="Cermenati, Solei" sort="Cermenati, Solei" uniqKey="Cermenati S" first="Solei" last="Cermenati">Solei Cermenati</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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