Vascular Endothelial (VE)-Cadherin, Endothelial Adherens Junctions, and Vascular Disease

Lampugnani, Maria GraziaInst Mol Oncol, FIRC, I-20139 Milan, Italy;Mario Negri Inst Pharmacol Res, I-20156 Milan, Italy (author)

Endothelial cell - cell adherens junctions (AJs) supervise fundamental vascular functions, such as the control of permeability and transmigration of circulating leukocytes, and the maintenance of existing vessels and formation of new ones. These processes are often dysregulated in pathologies. However, the evidence that links dysfunction of endothelial AJs to human pathologies is mostly correlative. In this review, we present an update of the molecular organization of AJ complexes in endothelial cells (ECs) that is mainly based on observations from experimental models. Furthermore, we report in detail on a human pathology, cerebral cavernous malformation (CCM), which is initiated by loss-of-function mutations in the genes that encode the three cytoplasmic components of AJs (CCM1, CCM2, and CCM3). At present, these represent a unique example of mutations in components of endothelial AJs that cause human disease. We describe also how studies into the defects of AJs in CCM are shedding light on the crucial regulatory mechanisms and signaling activities of these endothelial structures. Although these observations are specific for CCM, they support the concept that dysfunction of endothelial AJs can directly contribute to human pathologies.

Dejana, ElisabettaUppsala universitet,Vaskulärbiologi,Inst Mol Oncol, FIRC, I-20139 Milan, Italy(Swepub:uu)elide443 (author)
Giampietro, CostanzaInst Mol Oncol, FIRC, I-20139 Milan, Italy (author)
Inst Mol Oncol, FIRC, I-20139 Milan, Italy;Mario Negri Inst Pharmacol Res, I-20156 Milan, ItalyVaskulärbiologi (creator_code:org_t)

In:Cold Spring Harbor Perspectives in Biology: COLD SPRING HARBOR LAB PRESS, PUBLICATIONS DEPT10:101943-0264

By the author/editor: Lampugnani, Mari ...; Dejana, Elisabet ...; Giampietro, Cost ...

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Basic Medicine; and Cell and Molecul ...

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