Disruption of acvrl1 increases endothelial cell number in zebrafish cranial vessels.
Disruption of acvrl1 increases endothelial cell number in zebrafish cranial vessels.
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发表时间：
2002-06
期刊：
影响因子：
4.6
通讯作者：
B. L. Roman;V. N. Pham;N. Lawson;Magdalena Kulik;S. Childs;A. Lekven;D. Garrity;R. Moon;M. Fishman
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作者：
B. L. Roman;V. N. Pham;N. Lawson;Magdalena Kulik;S. Childs;A. Lekven;D. Garrity;R. Moon;M. Fishman
斑马鱼突变体violet beautiflude（vbg）在受精后两天可以通过异常循环模式识别，其中大多数血细胞流经有限数量的扩张的颅血管，无法灌注躯干和尾部。这种表型不能用尾血管异常或颅血管图案缺陷来解释，而是源于特定颅血管中内皮细胞数量的增加。我们发现，vbg编码激活素受体样激酶1（Acvrl 1;也称为Alk 1），一种TGF β I型受体，主要表达于血管内皮细胞，在vbg突变体中扩张。因此，vbg为人类常染色体显性遗传性疾病遗传性出血性毛细血管扩张症2型提供了模型，其中ACVRL 1的破坏导致可能导致出血或中风的血管畸形。在线提供电影
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 TGFbeta 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. Movies available on-line