作者
Beth L Roman, Van N Pham, Nathan D Lawson, Magdalena Kulik, Sarah Childs, Arne C Lekven, Deborah M Garrity, Randall T Moon, Mark C Fishman, Robert J Lechleider, Brant M Weinstein
发表日期
2002/6/15
期刊
Development
卷号
129
期号
12
页码范围
3009-3019
出版商
The Company of Biologists Ltd
简介
The zebrafish mutant violet beauregarde (vbg) can be identified at two days post-fertilization by an abnormal circulation pattern in which most blood cells flow through a limited number of dilated cranial vessels and fail to perfuse the trunk and tail. This phenotype cannot be explained by caudal vessel abnormalities or by a defect in cranial vessel patterning, but instead stems from an increase in endothelial cell number in specific cranial vessels. We show that vbg encodes activin receptor-like kinase 1 (Acvrl1; also known as Alk1), a TGFβ type I receptor that is expressed predominantly in the endothelium of the vessels that become dilated in vbg mutants. Thus, vbg provides a model for the human autosomal dominant disorder, hereditary hemorrhagic telangiectasia type 2, in which disruption of ACVRL1 causes vessel malformations that may result in hemorrhage or stroke.
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BL Roman, VN Pham, ND Lawson, M Kulik, S Childs… - 2002