LymphedemaV1, Main, Exploration, bibRecord, 006C50

Emilin1 Deficiency Causes Structural and Functional Defects of Lymphatic Vasculature ▿

Identifieur interne : 006C50 ( Main/Exploration ); précédent : 006C49; suivant : 006C51

Emilin1 Deficiency Causes Structural and Functional Defects of Lymphatic Vasculature ▿

Auteurs : Carla Danussi ; Paola Spessotto ; Alessandra Petrucco ; Bruna Wassermann ; Patrizia Sabatelli ; Monica Montesi ; Roberto Doliana ; Giorgio M. Bressan ; Alfonso Colombatti

Source :

Molecular and Cellular Biology [ 0270-7306 ] ; 2008.

RBID : PMC:2423131

Descripteurs français

KwdFr :
- Animaux, Cellules cultivées, Glycoprotéines membranaires (physiologie), Humains, Lymphangiogenèse, Matrice extracellulaire (métabolisme), Modèles biologiques, Phénotype, Régulation de l'expression des gènes, Souris, Souris de lignée BALB C, Souris de lignée C57BL, Souris transgéniques, Vaisseaux lymphatiques (anatomopathologie), Vaisseaux lymphatiques (métabolisme).
MESH :
- anatomopathologie : Vaisseaux lymphatiques.
- métabolisme : Matrice extracellulaire, Vaisseaux lymphatiques.
- physiologie : Glycoprotéines membranaires.
- Animaux, Cellules cultivées, Humains, Lymphangiogenèse, Modèles biologiques, Phénotype, Régulation de l'expression des gènes, Souris, Souris de lignée BALB C, Souris de lignée C57BL, Souris transgéniques.

English descriptors

KwdEn :
- Animals, Cells, Cultured, Extracellular Matrix (metabolism), Gene Expression Regulation, Humans, Lymphangiogenesis, Lymphatic Vessels (metabolism), Lymphatic Vessels (pathology), Membrane Glycoproteins (physiology), Mice, Mice, Inbred BALB C, Mice, Inbred C57BL, Mice, Transgenic, Models, Biological, Phenotype.
MESH :
- chemical , physiology : Membrane Glycoproteins.
- metabolism : Extracellular Matrix, Lymphatic Vessels.
- pathology : Lymphatic Vessels.
- Animals, Cells, Cultured, Gene Expression Regulation, Humans, Lymphangiogenesis, Mice, Mice, Inbred BALB C, Mice, Inbred C57BL, Mice, Transgenic, Models, Biological, Phenotype.

Abstract

Lymphatic-vasculature function critically depends on extracellular matrix (ECM) and on its connections with lymphatic endothelial cells (LECs). However, the composition and the architecture of ECM have not been fully taken into consideration in studying the biology and the pathology of the lymphatic system. EMILIN1, an elastic microfibril-associated protein, is highly expressed by LECs in vitro and colocalizes with lymphatic vessels in several mouse tissues. A comparative study between WT and Emilin1^−/− mice highlighted the fact that Emilin1 deficiency in both CD1 and C57BL/6 backgrounds results in hyperplasia, enlargement, and frequently an irregular pattern of superficial and visceral lymphatic vessels and in a significant reduction of anchoring filaments. Emilin1-deficient mice also develop larger lymphangiomas than WT mice. Lymphatic vascular morphological alterations are accompanied by functional defects, such as mild lymphedema, a highly significant drop in lymph drainage, and enhanced lymph leakage. Our findings demonstrate that EMILIN1 is involved in the regulation of the growth and in the maintenance of the integrity of lymphatic vessels, a fundamental requirement for efficient function. The phenotype displayed by Emilin1^−/− mice is the first abnormal lymphatic phenotype associated with the deficiency of an ECM protein and identifies EMILIN1 as a novel local regulator of lymphangiogenesis.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2423131

DOI: 10.1128/MCB.02062-07
PubMed: 18411305
PubMed Central: 2423131

Affiliations:

Le document en format XML

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<term>Humans</term>
<term>Lymphangiogenesis</term>
<term>Lymphatic Vessels (metabolism)</term>
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<term>Membrane Glycoproteins (physiology)</term>
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<term>Glycoprotéines membranaires (physiologie)</term>
<term>Humains</term>
<term>Lymphangiogenèse</term>
<term>Matrice extracellulaire (métabolisme)</term>
<term>Modèles biologiques</term>
<term>Phénotype</term>
<term>Régulation de l'expression des gènes</term>
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<term>Souris de lignée BALB C</term>
<term>Souris de lignée C57BL</term>
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<term>Vaisseaux lymphatiques (métabolisme)</term>
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<term>Lymphatic Vessels</term>
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<term>Vaisseaux lymphatiques</term>
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<front><div type="abstract" xml:lang="en"><p>Lymphatic-vasculature function critically depends on extracellular matrix (ECM) and on its connections with lymphatic endothelial cells (LECs). However, the composition and the architecture of ECM have not been fully taken into consideration in studying the biology and the pathology of the lymphatic system. EMILIN1, an elastic microfibril-associated protein, is highly expressed by LECs in vitro and colocalizes with lymphatic vessels in several mouse tissues. A comparative study between WT and <italic>Emilin1</italic>
<sup>−/−</sup>
 mice highlighted the fact that <italic>Emilin1</italic>
 deficiency in both CD1 and C57BL/6 backgrounds results in hyperplasia, enlargement, and frequently an irregular pattern of superficial and visceral lymphatic vessels and in a significant reduction of anchoring filaments. <italic>Emilin1</italic>
-deficient mice also develop larger lymphangiomas than WT mice. Lymphatic vascular morphological alterations are accompanied by functional defects, such as mild lymphedema, a highly significant drop in lymph drainage, and enhanced lymph leakage. Our findings demonstrate that EMILIN1 is involved in the regulation of the growth and in the maintenance of the integrity of lymphatic vessels, a fundamental requirement for efficient function. The phenotype displayed by <italic>Emilin1</italic>
<sup>−/−</sup>
 mice is the first abnormal lymphatic phenotype associated with the deficiency of an ECM protein and identifies EMILIN1 as a novel local regulator of lymphangiogenesis.</p>
</div>
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<name sortKey="Colombatti, Alfonso" sort="Colombatti, Alfonso" uniqKey="Colombatti A" first="Alfonso" last="Colombatti">Alfonso Colombatti</name>
<name sortKey="Danussi, Carla" sort="Danussi, Carla" uniqKey="Danussi C" first="Carla" last="Danussi">Carla Danussi</name>
<name sortKey="Doliana, Roberto" sort="Doliana, Roberto" uniqKey="Doliana R" first="Roberto" last="Doliana">Roberto Doliana</name>
<name sortKey="Montesi, Monica" sort="Montesi, Monica" uniqKey="Montesi M" first="Monica" last="Montesi">Monica Montesi</name>
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<name sortKey="Wassermann, Bruna" sort="Wassermann, Bruna" uniqKey="Wassermann B" first="Bruna" last="Wassermann">Bruna Wassermann</name>
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Serveur d'exploration sur le lymphœdème

Emilin1 Deficiency Causes Structural and Functional Defects of Lymphatic Vasculature ▿

Emilin1 Deficiency Causes Structural and Functional Defects of Lymphatic Vasculature ▿

Source :

Descripteurs français

English descriptors

Abstract

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

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

Pour générer des pages wiki

	Serveur d'exploration sur le lymphœdème
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