LymphedemaV1, PubMed, Checkpoint, bibRecord, 000F11

Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.

Identifieur interne : 000F11 ( PubMed/Checkpoint ); précédent : 000F10; suivant : 000F12

Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.

Auteurs : M Guy Roukens ; Josi Peterson-Maduro ; Yvonne Padberg ; Michael Jeltsch ; Veli-Matti Lepp Nen ; Frank L. Bos ; Kari Alitalo [Finlande] ; Stefan Schulte-Merker ; Dörte Schulte [Allemagne]

Source :

Circulation research [ 1524-4571 ] ; 2015.

RBID : pubmed:25814692

Descripteurs français

English descriptors

KwdEn :
- Animals, Binding Sites, Calcium-Binding Proteins (chemistry), Calcium-Binding Proteins (deficiency), Calcium-Binding Proteins (genetics), Calcium-Binding Proteins (metabolism), Collagen (metabolism), Craniofacial Abnormalities (genetics), Craniofacial Abnormalities (metabolism), Endothelial Cells (metabolism), Epidermal Growth Factor (metabolism), Gene Expression Regulation, Developmental, Gene Knock-In Techniques, Genital Diseases, Male (genetics), Genital Diseases, Male (metabolism), Genotype, Gestational Age, HEK293 Cells, Humans, Lymphangiectasis, Intestinal (genetics), Lymphangiectasis, Intestinal (metabolism), Lymphatic Vessels (embryology), Lymphatic Vessels (metabolism), Lymphedema (genetics), Lymphedema (metabolism), Mice, Mice, Transgenic, Mutation, Phenotype, Protein Binding, Protein Interaction Domains and Motifs, Signal Transduction, Transfection, Tumor Suppressor Proteins (chemistry), Tumor Suppressor Proteins (deficiency), Tumor Suppressor Proteins (genetics), Tumor Suppressor Proteins (metabolism), Vascular Endothelial Growth Factor C (metabolism), Zebrafish (genetics), Zebrafish (metabolism), Zebrafish Proteins (chemistry), Zebrafish Proteins (genetics), Zebrafish Proteins (metabolism).
MESH :
- chemical , chemistry : Calcium-Binding Proteins, Tumor Suppressor Proteins, Zebrafish Proteins.
- chemical , deficiency : Calcium-Binding Proteins, Tumor Suppressor Proteins.
- chemical , genetics : Calcium-Binding Proteins, Tumor Suppressor Proteins, Zebrafish Proteins.
- chemical , metabolism : Calcium-Binding Proteins, Collagen, Epidermal Growth Factor, Tumor Suppressor Proteins, Vascular Endothelial Growth Factor C, Zebrafish Proteins.
- embryology : Lymphatic Vessels.
- genetics : Craniofacial Abnormalities, Genital Diseases, Male, Lymphangiectasis, Intestinal, Lymphedema, Zebrafish.
- metabolism : Craniofacial Abnormalities, Endothelial Cells, Genital Diseases, Male, Lymphangiectasis, Intestinal, Lymphatic Vessels, Lymphedema, Zebrafish.
- Animals, Binding Sites, Gene Expression Regulation, Developmental, Gene Knock-In Techniques, Genotype, Gestational Age, HEK293 Cells, Humans, Mice, Mice, Transgenic, Mutation, Phenotype, Protein Binding, Protein Interaction Domains and Motifs, Signal Transduction, Transfection.

Abstract

Collagen- and calcium-binding EGF domain-containing protein 1 (CCBE1) is essential for lymphangiogenesis in vertebrates and has been associated with Hennekam syndrome. Recently, CCBE1 has emerged as a crucial regulator of vascular endothelial growth factor-C (VEGFC) signaling.

DOI: 10.1161/CIRCRESAHA.116.304949
PubMed: 25814692

Affiliations:

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

to stream PubMed, to step Corpus: 000F58
to stream PubMed, to step Curation: 000F58

Links to Exploration step

pubmed:25814692

Le document en format XML

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<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Peterson Maduro, Josi" sort="Peterson Maduro, Josi" uniqKey="Peterson Maduro J" first="Josi" last="Peterson-Maduro">Josi Peterson-Maduro</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Padberg, Yvonne" sort="Padberg, Yvonne" uniqKey="Padberg Y" first="Yvonne" last="Padberg">Yvonne Padberg</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Jeltsch, Michael" sort="Jeltsch, Michael" uniqKey="Jeltsch M" first="Michael" last="Jeltsch">Michael Jeltsch</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Lepp Nen, Veli Matti" sort="Lepp Nen, Veli Matti" uniqKey="Lepp Nen V" first="Veli-Matti" last="Lepp Nen">Veli-Matti Lepp Nen</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Bos, Frank L" sort="Bos, Frank L" uniqKey="Bos F" first="Frank L" last="Bos">Frank L. Bos</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Alitalo, Kari" sort="Alitalo, Kari" uniqKey="Alitalo K" first="Kari" last="Alitalo">Kari Alitalo</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
<country>Finlande</country>
<placeName><settlement type="city">Helsinki</settlement>
<region type="région" nuts="2">Uusimaa</region>
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<orgName type="university">Université d'Helsinki</orgName>
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<author><name sortKey="Schulte Merker, Stefan" sort="Schulte Merker, Stefan" uniqKey="Schulte Merker S" first="Stefan" last="Schulte-Merker">Stefan Schulte-Merker</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Schulte, Dorte" sort="Schulte, Dorte" uniqKey="Schulte D" first="Dörte" last="Schulte">Dörte Schulte</name>
<affiliation wicri:level="1"><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.). d.schulte@uni-muenster.de.</nlm:affiliation>
<country wicri:rule="url">Allemagne</country>
</affiliation>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.</title>
<author><name sortKey="Roukens, M Guy" sort="Roukens, M Guy" uniqKey="Roukens M" first="M Guy" last="Roukens">M Guy Roukens</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
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<author><name sortKey="Peterson Maduro, Josi" sort="Peterson Maduro, Josi" uniqKey="Peterson Maduro J" first="Josi" last="Peterson-Maduro">Josi Peterson-Maduro</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
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<author><name sortKey="Padberg, Yvonne" sort="Padberg, Yvonne" uniqKey="Padberg Y" first="Yvonne" last="Padberg">Yvonne Padberg</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
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<author><name sortKey="Jeltsch, Michael" sort="Jeltsch, Michael" uniqKey="Jeltsch M" first="Michael" last="Jeltsch">Michael Jeltsch</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Lepp Nen, Veli Matti" sort="Lepp Nen, Veli Matti" uniqKey="Lepp Nen V" first="Veli-Matti" last="Lepp Nen">Veli-Matti Lepp Nen</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Bos, Frank L" sort="Bos, Frank L" uniqKey="Bos F" first="Frank L" last="Bos">Frank L. Bos</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Alitalo, Kari" sort="Alitalo, Kari" uniqKey="Alitalo K" first="Kari" last="Alitalo">Kari Alitalo</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
<country>Finlande</country>
<placeName><settlement type="city">Helsinki</settlement>
<region type="région" nuts="2">Uusimaa</region>
</placeName>
<orgName type="university">Université d'Helsinki</orgName>
</affiliation>
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<author><name sortKey="Schulte Merker, Stefan" sort="Schulte Merker, Stefan" uniqKey="Schulte Merker S" first="Stefan" last="Schulte-Merker">Stefan Schulte-Merker</name>
<affiliation><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</nlm:affiliation>
<wicri:noCountry code="subField">D.S.).</wicri:noCountry>
</affiliation>
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<author><name sortKey="Schulte, Dorte" sort="Schulte, Dorte" uniqKey="Schulte D" first="Dörte" last="Schulte">Dörte Schulte</name>
<affiliation wicri:level="1"><nlm:affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.). d.schulte@uni-muenster.de.</nlm:affiliation>
<country wicri:rule="url">Allemagne</country>
</affiliation>
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</analytic>
<series><title level="j">Circulation research</title>
<idno type="eISSN">1524-4571</idno>
<imprint><date when="2015" type="published">2015</date>
</imprint>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Animals</term>
<term>Binding Sites</term>
<term>Calcium-Binding Proteins (chemistry)</term>
<term>Calcium-Binding Proteins (deficiency)</term>
<term>Calcium-Binding Proteins (genetics)</term>
<term>Calcium-Binding Proteins (metabolism)</term>
<term>Collagen (metabolism)</term>
<term>Craniofacial Abnormalities (genetics)</term>
<term>Craniofacial Abnormalities (metabolism)</term>
<term>Endothelial Cells (metabolism)</term>
<term>Epidermal Growth Factor (metabolism)</term>
<term>Gene Expression Regulation, Developmental</term>
<term>Gene Knock-In Techniques</term>
<term>Genital Diseases, Male (genetics)</term>
<term>Genital Diseases, Male (metabolism)</term>
<term>Genotype</term>
<term>Gestational Age</term>
<term>HEK293 Cells</term>
<term>Humans</term>
<term>Lymphangiectasis, Intestinal (genetics)</term>
<term>Lymphangiectasis, Intestinal (metabolism)</term>
<term>Lymphatic Vessels (embryology)</term>
<term>Lymphatic Vessels (metabolism)</term>
<term>Lymphedema (genetics)</term>
<term>Lymphedema (metabolism)</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Mutation</term>
<term>Phenotype</term>
<term>Protein Binding</term>
<term>Protein Interaction Domains and Motifs</term>
<term>Signal Transduction</term>
<term>Transfection</term>
<term>Tumor Suppressor Proteins (chemistry)</term>
<term>Tumor Suppressor Proteins (deficiency)</term>
<term>Tumor Suppressor Proteins (genetics)</term>
<term>Tumor Suppressor Proteins (metabolism)</term>
<term>Vascular Endothelial Growth Factor C (metabolism)</term>
<term>Zebrafish (genetics)</term>
<term>Zebrafish (metabolism)</term>
<term>Zebrafish Proteins (chemistry)</term>
<term>Zebrafish Proteins (genetics)</term>
<term>Zebrafish Proteins (metabolism)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Animaux</term>
<term>Cellules HEK293</term>
<term>Cellules endothéliales (métabolisme)</term>
<term>Collagène (métabolisme)</term>
<term>Danio zébré (génétique)</term>
<term>Danio zébré (métabolisme)</term>
<term>Facteur de croissance endothéliale vasculaire de type C (métabolisme)</term>
<term>Facteur de croissance épidermique (métabolisme)</term>
<term>Génotype</term>
<term>Humains</term>
<term>Liaison aux protéines</term>
<term>Lymphangiectasie intestinale (génétique)</term>
<term>Lymphangiectasie intestinale (métabolisme)</term>
<term>Lymphoedème (génétique)</term>
<term>Lymphoedème (métabolisme)</term>
<term>Maladies de l'appareil génital mâle (génétique)</term>
<term>Maladies de l'appareil génital mâle (métabolisme)</term>
<term>Malformations crâniofaciales (génétique)</term>
<term>Malformations crâniofaciales (métabolisme)</term>
<term>Motifs et domaines d'intéraction protéique</term>
<term>Mutation</term>
<term>Phénotype</term>
<term>Protéines de liaison au calcium ()</term>
<term>Protéines de liaison au calcium (déficit)</term>
<term>Protéines de liaison au calcium (génétique)</term>
<term>Protéines de liaison au calcium (métabolisme)</term>
<term>Protéines de poisson-zèbre ()</term>
<term>Protéines de poisson-zèbre (génétique)</term>
<term>Protéines de poisson-zèbre (métabolisme)</term>
<term>Protéines suppresseurs de tumeurs ()</term>
<term>Protéines suppresseurs de tumeurs (déficit)</term>
<term>Protéines suppresseurs de tumeurs (génétique)</term>
<term>Protéines suppresseurs de tumeurs (métabolisme)</term>
<term>Régulation de l'expression des gènes au cours du développement</term>
<term>Sites de fixation</term>
<term>Souris</term>
<term>Souris transgéniques</term>
<term>Techniques de knock-in de gènes</term>
<term>Transduction du signal</term>
<term>Transfection</term>
<term>Vaisseaux lymphatiques (embryologie)</term>
<term>Vaisseaux lymphatiques (métabolisme)</term>
<term>Âge gestationnel</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="chemistry" xml:lang="en"><term>Calcium-Binding Proteins</term>
<term>Tumor Suppressor Proteins</term>
<term>Zebrafish Proteins</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="deficiency" xml:lang="en"><term>Calcium-Binding Proteins</term>
<term>Tumor Suppressor Proteins</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Calcium-Binding Proteins</term>
<term>Tumor Suppressor Proteins</term>
<term>Zebrafish Proteins</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Calcium-Binding Proteins</term>
<term>Collagen</term>
<term>Epidermal Growth Factor</term>
<term>Tumor Suppressor Proteins</term>
<term>Vascular Endothelial Growth Factor C</term>
<term>Zebrafish Proteins</term>
</keywords>
<keywords scheme="MESH" qualifier="déficit" xml:lang="fr"><term>Protéines de liaison au calcium</term>
<term>Protéines suppresseurs de tumeurs</term>
</keywords>
<keywords scheme="MESH" qualifier="embryologie" xml:lang="fr"><term>Vaisseaux lymphatiques</term>
</keywords>
<keywords scheme="MESH" qualifier="embryology" xml:lang="en"><term>Lymphatic Vessels</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Craniofacial Abnormalities</term>
<term>Genital Diseases, Male</term>
<term>Lymphangiectasis, Intestinal</term>
<term>Lymphedema</term>
<term>Zebrafish</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Danio zébré</term>
<term>Lymphangiectasie intestinale</term>
<term>Lymphoedème</term>
<term>Maladies de l'appareil génital mâle</term>
<term>Malformations crâniofaciales</term>
<term>Protéines de liaison au calcium</term>
<term>Protéines de poisson-zèbre</term>
<term>Protéines suppresseurs de tumeurs</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Craniofacial Abnormalities</term>
<term>Endothelial Cells</term>
<term>Genital Diseases, Male</term>
<term>Lymphangiectasis, Intestinal</term>
<term>Lymphatic Vessels</term>
<term>Lymphedema</term>
<term>Zebrafish</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Cellules endothéliales</term>
<term>Collagène</term>
<term>Danio zébré</term>
<term>Facteur de croissance endothéliale vasculaire de type C</term>
<term>Facteur de croissance épidermique</term>
<term>Lymphangiectasie intestinale</term>
<term>Lymphoedème</term>
<term>Maladies de l'appareil génital mâle</term>
<term>Malformations crâniofaciales</term>
<term>Protéines de liaison au calcium</term>
<term>Protéines de poisson-zèbre</term>
<term>Protéines suppresseurs de tumeurs</term>
<term>Vaisseaux lymphatiques</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Animals</term>
<term>Binding Sites</term>
<term>Gene Expression Regulation, Developmental</term>
<term>Gene Knock-In Techniques</term>
<term>Genotype</term>
<term>Gestational Age</term>
<term>HEK293 Cells</term>
<term>Humans</term>
<term>Mice</term>
<term>Mice, Transgenic</term>
<term>Mutation</term>
<term>Phenotype</term>
<term>Protein Binding</term>
<term>Protein Interaction Domains and Motifs</term>
<term>Signal Transduction</term>
<term>Transfection</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Animaux</term>
<term>Cellules HEK293</term>
<term>Génotype</term>
<term>Humains</term>
<term>Liaison aux protéines</term>
<term>Motifs et domaines d'intéraction protéique</term>
<term>Mutation</term>
<term>Phénotype</term>
<term>Protéines de liaison au calcium</term>
<term>Protéines de poisson-zèbre</term>
<term>Protéines suppresseurs de tumeurs</term>
<term>Régulation de l'expression des gènes au cours du développement</term>
<term>Sites de fixation</term>
<term>Souris</term>
<term>Souris transgéniques</term>
<term>Techniques de knock-in de gènes</term>
<term>Transduction du signal</term>
<term>Transfection</term>
<term>Âge gestationnel</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Collagen- and calcium-binding EGF domain-containing protein 1 (CCBE1) is essential for lymphangiogenesis in vertebrates and has been associated with Hennekam syndrome. Recently, CCBE1 has emerged as a crucial regulator of vascular endothelial growth factor-C (VEGFC) signaling.</div>
</front>
</TEI>
<pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">25814692</PMID>
<DateCreated><Year>2015</Year>
<Month>05</Month>
<Day>08</Day>
</DateCreated>
<DateCompleted><Year>2015</Year>
<Month>07</Month>
<Day>21</Day>
</DateCompleted>
<DateRevised><Year>2015</Year>
<Month>05</Month>
<Day>08</Day>
</DateRevised>
<Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1524-4571</ISSN>
<JournalIssue CitedMedium="Internet"><Volume>116</Volume>
<Issue>10</Issue>
<PubDate><Year>2015</Year>
<Month>May</Month>
<Day>08</Day>
</PubDate>
</JournalIssue>
<Title>Circulation research</Title>
<ISOAbbreviation>Circ. Res.</ISOAbbreviation>
</Journal>
<ArticleTitle>Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.</ArticleTitle>
<Pagination><MedlinePgn>1660-9</MedlinePgn>
</Pagination>
<ELocationID EIdType="doi" ValidYN="Y">10.1161/CIRCRESAHA.116.304949</ELocationID>
<Abstract><AbstractText Label="RATIONALE" NlmCategory="BACKGROUND">Collagen- and calcium-binding EGF domain-containing protein 1 (CCBE1) is essential for lymphangiogenesis in vertebrates and has been associated with Hennekam syndrome. Recently, CCBE1 has emerged as a crucial regulator of vascular endothelial growth factor-C (VEGFC) signaling.</AbstractText>
<AbstractText Label="OBJECTIVE" NlmCategory="OBJECTIVE">CCBE1 is a secreted protein characterized by 2 EGF domains and 2 collagen repeats. The functional role of the different CCBE1 protein domains is completely unknown. Here, we analyzed the functional role of the different CCBE1 domains in vivo and in vitro.</AbstractText>
<AbstractText Label="METHODS AND RESULTS" NlmCategory="RESULTS">We analyzed the functionality of several CCBE1 deletion mutants by generating knock-in mice expressing these mutants, by analyzing their ability to enhance Vegfc signaling in vivo in zebrafish, and by testing their ability to induce VEGFC processing in vitro. We found that deleting the collagen domains of CCBE1 has a much stronger effect on CCBE1 activity than deleting the EGF domains. First, although CCBE1ΔCollagen mice fully phenocopy CCBE1 knock-out mice, CCBE1ΔEGF knock-in embryos still form rudimentary lymphatics. Second, Ccbe1ΔEGF, but not Ccbe1ΔCollagen, could partially substitute for Ccbe1 to enhance Vegfc signaling in zebrafish. Third, CCBE1ΔEGF, similarly to CCBE1, but not CCBE1ΔCollagen could activate VEGFC processing in vitro. Furthermore, a Hennekam syndrome mutation within the collagen domain has a stronger effect than a Hennekam syndrome mutation within the EGF domain.</AbstractText>
<AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">We propose that the collagen domains of CCBE1 are crucial for the activation of VEGFC in vitro and in vivo. The EGF domains of CCBE1 are dispensable for regulation of VEGFC processing in vitro, however, they are necessary for full lymphangiogenic activity of CCBE1 in vivo.</AbstractText>
<CopyrightInformation>© 2015 American Heart Association, Inc.</CopyrightInformation>
</Abstract>
<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Roukens</LastName>
<ForeName>M Guy</ForeName>
<Initials>MG</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Peterson-Maduro</LastName>
<ForeName>Josi</ForeName>
<Initials>J</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Padberg</LastName>
<ForeName>Yvonne</ForeName>
<Initials>Y</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Jeltsch</LastName>
<ForeName>Michael</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Leppänen</LastName>
<ForeName>Veli-Matti</ForeName>
<Initials>VM</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Bos</LastName>
<ForeName>Frank L</ForeName>
<Initials>FL</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Alitalo</LastName>
<ForeName>Kari</ForeName>
<Initials>K</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Schulte-Merker</LastName>
<ForeName>Stefan</ForeName>
<Initials>S</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.).</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Schulte</LastName>
<ForeName>Dörte</ForeName>
<Initials>D</Initials>
<AffiliationInfo><Affiliation>From the Hubrecht Institute, KNAW-UMC Utrecht, Utrecht, The Netherlands (M.G.R., J.P.M., Y.P., F.L.B., S.S.-M., D.S.); Cardiovascular Research Institute, University of California San Francisco (F.L.B.); Translational Cancer Biology Program, University of Helsinki, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Wihuri Research Institute, Biomedicum Helsinki, Helsinki, Finland (M.J., V.-M. L., K.A.); Institute for Cardiovascular Organogenesis and Regeneration, Faculty of Medicine, WWU, Münster, Germany (Y.P., S.S.M., D.S.); and Cells-in-Motion Cluster of Excellence (EXC 1003-CiM), University of Münster, Münster, Germany (Y.P., S.S.M., D.S.). d.schulte@uni-muenster.de.</Affiliation>
</AffiliationInfo>
</Author>
</AuthorList>
<Language>eng</Language>
<PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType>
<PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType>
</PublicationTypeList>
<ArticleDate DateType="Electronic"><Year>2015</Year>
<Month>03</Month>
<Day>26</Day>
</ArticleDate>
</Article>
<MedlineJournalInfo><Country>United States</Country>
<MedlineTA>Circ Res</MedlineTA>
<NlmUniqueID>0047103</NlmUniqueID>
<ISSNLinking>0009-7330</ISSNLinking>
</MedlineJournalInfo>
<ChemicalList><Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C546964">CCBE1 protein, human</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C000599480">CCBE1 protein, zebrafish</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D002135">Calcium-Binding Proteins</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C572375">Ccbe1 protein, mouse</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D025521">Tumor Suppressor Proteins</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C467486">VEGFC protein, human</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D042582">Vascular Endothelial Growth Factor C</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D029961">Zebrafish Proteins</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C467483">vascular endothelial growth factor C, mouse</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="C493178">vascular endothelial growth factor C, zebrafish</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>62229-50-9</RegistryNumber>
<NameOfSubstance UI="D004815">Epidermal Growth Factor</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>9007-34-5</RegistryNumber>
<NameOfSubstance UI="D003094">Collagen</NameOfSubstance>
</Chemical>
</ChemicalList>
<SupplMeshList><SupplMeshName Type="Disease" UI="C537255">Hennekam lymphangiectasia lymphedema syndrome</SupplMeshName>
</SupplMeshList>
<CitationSubset>IM</CitationSubset>
<MeshHeadingList><MeshHeading><DescriptorName UI="D000818" MajorTopicYN="N">Animals</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D001665" MajorTopicYN="N">Binding Sites</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D002135" MajorTopicYN="N">Calcium-Binding Proteins</DescriptorName>
<QualifierName UI="Q000737" MajorTopicYN="N">chemistry</QualifierName>
<QualifierName UI="Q000172" MajorTopicYN="N">deficiency</QualifierName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D003094" MajorTopicYN="N">Collagen</DescriptorName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D019465" MajorTopicYN="N">Craniofacial Abnormalities</DescriptorName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D042783" MajorTopicYN="N">Endothelial Cells</DescriptorName>
<QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D004815" MajorTopicYN="N">Epidermal Growth Factor</DescriptorName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D018507" MajorTopicYN="N">Gene Expression Regulation, Developmental</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D055879" MajorTopicYN="N">Gene Knock-In Techniques</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D005832" MajorTopicYN="N">Genital Diseases, Male</DescriptorName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D005838" MajorTopicYN="N">Genotype</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D005865" MajorTopicYN="N">Gestational Age</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D057809" MajorTopicYN="N">HEK293 Cells</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008201" MajorTopicYN="N">Lymphangiectasis, Intestinal</DescriptorName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D042601" MajorTopicYN="N">Lymphatic Vessels</DescriptorName>
<QualifierName UI="Q000196" MajorTopicYN="N">embryology</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D051379" MajorTopicYN="N">Mice</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008822" MajorTopicYN="N">Mice, Transgenic</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D009154" MajorTopicYN="N">Mutation</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D010641" MajorTopicYN="N">Phenotype</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D011485" MajorTopicYN="N">Protein Binding</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D054730" MajorTopicYN="N">Protein Interaction Domains and Motifs</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D015398" MajorTopicYN="N">Signal Transduction</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D014162" MajorTopicYN="N">Transfection</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D025521" MajorTopicYN="N">Tumor Suppressor Proteins</DescriptorName>
<QualifierName UI="Q000737" MajorTopicYN="N">chemistry</QualifierName>
<QualifierName UI="Q000172" MajorTopicYN="N">deficiency</QualifierName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D042582" MajorTopicYN="N">Vascular Endothelial Growth Factor C</DescriptorName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D015027" MajorTopicYN="N">Zebrafish</DescriptorName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D029961" MajorTopicYN="N">Zebrafish Proteins</DescriptorName>
<QualifierName UI="Q000737" MajorTopicYN="N">chemistry</QualifierName>
<QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName>
<QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName>
</MeshHeading>
</MeshHeadingList>
<KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">CCBE1 protein</Keyword>
<Keyword MajorTopicYN="N">Hennekam lymphangiectasia-lymphedema syndrome</Keyword>
<Keyword MajorTopicYN="N">endothelium, vascular</Keyword>
<Keyword MajorTopicYN="N">lymphangiogenesis</Keyword>
<Keyword MajorTopicYN="N">vascular endothelial growth factor</Keyword>
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</MedlineCitation>
<PubmedData><History><PubMedPubDate PubStatus="received"><Year>2014</Year>
<Month>07</Month>
<Day>31</Day>
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<PubMedPubDate PubStatus="accepted"><Year>2015</Year>
<Month>03</Month>
<Day>26</Day>
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<PubMedPubDate PubStatus="entrez"><Year>2015</Year>
<Month>3</Month>
<Day>28</Day>
<Hour>6</Hour>
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<PubMedPubDate PubStatus="pubmed"><Year>2015</Year>
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<Day>31</Day>
<Hour>6</Hour>
<Minute>0</Minute>
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<PubMedPubDate PubStatus="medline"><Year>2015</Year>
<Month>7</Month>
<Day>22</Day>
<Hour>6</Hour>
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</History>
<PublicationStatus>ppublish</PublicationStatus>
<ArticleIdList><ArticleId IdType="pubmed">25814692</ArticleId>
<ArticleId IdType="pii">CIRCRESAHA.116.304949</ArticleId>
<ArticleId IdType="doi">10.1161/CIRCRESAHA.116.304949</ArticleId>
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</PubmedData>
</pubmed>
<affiliations><list><country><li>Allemagne</li>
<li>Finlande</li>
</country>
<region><li>Uusimaa</li>
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<settlement><li>Helsinki</li>
</settlement>
<orgName><li>Université d'Helsinki</li>
</orgName>
</list>
<tree><noCountry><name sortKey="Bos, Frank L" sort="Bos, Frank L" uniqKey="Bos F" first="Frank L" last="Bos">Frank L. Bos</name>
<name sortKey="Jeltsch, Michael" sort="Jeltsch, Michael" uniqKey="Jeltsch M" first="Michael" last="Jeltsch">Michael Jeltsch</name>
<name sortKey="Lepp Nen, Veli Matti" sort="Lepp Nen, Veli Matti" uniqKey="Lepp Nen V" first="Veli-Matti" last="Lepp Nen">Veli-Matti Lepp Nen</name>
<name sortKey="Padberg, Yvonne" sort="Padberg, Yvonne" uniqKey="Padberg Y" first="Yvonne" last="Padberg">Yvonne Padberg</name>
<name sortKey="Peterson Maduro, Josi" sort="Peterson Maduro, Josi" uniqKey="Peterson Maduro J" first="Josi" last="Peterson-Maduro">Josi Peterson-Maduro</name>
<name sortKey="Roukens, M Guy" sort="Roukens, M Guy" uniqKey="Roukens M" first="M Guy" last="Roukens">M Guy Roukens</name>
<name sortKey="Schulte Merker, Stefan" sort="Schulte Merker, Stefan" uniqKey="Schulte Merker S" first="Stefan" last="Schulte-Merker">Stefan Schulte-Merker</name>
</noCountry>
<country name="Finlande"><region name="Uusimaa"><name sortKey="Alitalo, Kari" sort="Alitalo, Kari" uniqKey="Alitalo K" first="Kari" last="Alitalo">Kari Alitalo</name>
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</country>
<country name="Allemagne"><noRegion><name sortKey="Schulte, Dorte" sort="Schulte, Dorte" uniqKey="Schulte D" first="Dörte" last="Schulte">Dörte Schulte</name>
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</record>

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Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.

Functional Dissection of the CCBE1 Protein: A Crucial Requirement for the Collagen Repeat Domain.

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