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- W104810769 abstract "ALK1 (Activin Receptor-Like Kinase 1) belongs to the type I receptor family for TGF-β family ligands. Heterozygous ALK1 mutations cause HHT2, a multisystemic vascular disorder, characterized by telangiectases and arteriovenous malformations (AVMs) in brain, lung, and visceral organs. Pathogenetic mechanisms underlying vascular malformations in HHT are poorly understood. To develop an animal model for HHT, we generated a Alk1 conditional knockout mouse line. Alk1 deletion in limited vascular endothelial cells by a novel endothelial Cre deleter line resulted in lethality by PN6 with massive hemorrhage in the lung. Numerous AVMs were detected in the cerebral and pulmonary vessels by corrosion casting and latex dye injections. The Alk1 mutants exhibited severe pulmonary vascular malformations mimicking all pathological features of HHT-dilation of lumen, thinning of vascular walls, loss of capillaries, development of excessive tortuous vessels, and AVMs. Microarray analysis on PN3 and PN4 lungs of the control and mutant mice revealed that expression of genes involved in biosynthesis, metabolism or signaling of multiple pathways regulating angiogenesis and vascular tone was altered. These pathways include nitric oxide, angiotensin, endothelin, prostaglandins and sphingosine-1-phosphate pathways, providing important clues to understanding the pathogenesis of HHT." @default.
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- W104810769 date "2008-03-01" @default.
- W104810769 modified "2023-09-23" @default.
- W104810769 title "ALK1 signaling plays a pivotal role in regulation of genes involved in angiogenesis and vascular tone: implication on the pathogenetic mechanism for hereditary hemorrhagic telangiectasia 2 (HHT2)" @default.
- W104810769 doi "https://doi.org/10.1096/fasebj.22.1_supplement.318.1" @default.
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