Coexistence of Eph receptor B1 and ephrin B2 in port-wine stain endothelial progenitor cells contributes to clinicopathological vasculature dilatation.
Coexistence of Eph receptor B1 and ephrin B2 in port-wine stain endothelial progenitor cells contributes to clinicopathological vasculature dilatation.
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DOI：
10.1111/bjd.15716
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发表时间：
2017-12
影响因子：
10.3
通讯作者：
Nelson, J. S.
中科院分区：
文献类型：
--
作者：
Tan, W.;Wang, J.;Zhou, F.;Gao, L.;Yin, R.;Liu, H.;Sukanthanag, A.;Wang, G.;Mihm, M. C., Jr.;Chen, D-B;Nelson, J. S.
葡萄酒港染色（PWS）是一种以毛细血管后小静脉进行性扩张为特征的血管畸形，但其分子发病机制尚不清楚。为了说明PWS内皮细胞（EC）呈现独特的分子表型，导致病理解剖PWS血管。免疫组化和透射电镜观察PWS血管的超微结构和分子表型。采用原代培养的人真皮微血管内皮细胞和体外管形成试验进行功能研究。PWS血管在疾病的发展和进展过程中表现出多种临床病理特征。PWS皮肤在表型和形态上没有观察到正常的小动脉和小静脉;小动脉和小静脉均表现出分化障碍，导致PWS病变中小动脉样血管减少和毛细血管环路形成缺陷。PWS ECs在非结节性病变中表现出干细胞特性，表达内皮祖细胞标志物CD 133和CD 166。它们还表达静脉/动脉双重身份，Eph受体B1（EphB 1）和肝配蛋白B2（EfnB 2）。EphB 1和EfnB 2在正常人真皮微血管内皮细胞中的共表达导致体外形成PWS样血管，例如较大直径和厚壁的毛细血管。PWS EC是分化受损的晚期内皮祖细胞，具有CD 133 +/CD 166 +/EphB 1 +/EfnB 2+的特定表型，其形成未成熟的小静脉样病理解剖血管。EphB 1和EfnB 2共存对正常EC-EC相互作用的破坏有助于PWS血管的进行性扩张。
Port-wine stain (PWS) is a vascular malformation characterized by progressive dilatation of postcapillary venules, but the molecular pathogenesis remains obscure. To illustrate that PWS endothelial cells (ECs) present a unique molecular phenotype that leads to pathoanatomical PWS vasculatures. Immunohistochemistry and transmission electron microscopy were used to characterize the ultrastructure and molecular phenotypes of PWS blood vessels. Primary culture of human dermal microvascular endothelial cells and in vitro tube formation assay were used for confirmative functional studies. Multiple clinicopathological features of PWS blood vessels during the development and progression of the disease were shown. There were no normal arterioles and venules observed phenotypically and morphologically in PWS skin; arterioles and venules both showed differentiation impairments, resulting in a reduction of arteriole-like vasculatures and defects in capillary loop formation in PWS lesions. PWS ECs showed stemness properties with expression of endothelial progenitor cell markers CD133 and CD166 in non-nodular lesions. They also expressed dual venous/arterial identities, Eph receptor B1 (EphB1) and ephrin B2 (EfnB2). Co-expression of EphB1 and EfnB2 in normal human dermal microvascular ECs led to the formation of PWS-like vasculatures in vitro, for example larger-diameter and thick-walled capillaries. PWS ECs are differentiation-impaired, late-stage endothelial progenitor cells with a specific phenotype of CD133+/CD166+/EphB1+/EfnB2+, which form immature venule-like pathoanatomical vasculatures. The disruption of normal EC–EC interactions by coexistence of EphB1 and EfnB2 contributes to progressive dilatation of PWS vasculatures.
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毛细血管畸形的内皮细胞富含体细胞 GNAQ 突变。
影响因子：
3.6
作者：
Couto JA;Huang L;Vivero MP;Kamitaki N;Maclellan RA;Mulliken JB;Bischoff J;Warman ML;Greene AK
通讯作者：
Greene AK
影响因子：
2.5
作者：
Li, Wenling;Mukouyama, Yoh-suke
通讯作者：
Mukouyama, Yoh-suke
影响因子：
7.3
作者：
Daar, Ira O.
通讯作者：
Daar, Ira O.
影响因子：
16
作者：
Gerety, SS;Wang, HU;Anderson, DJ
通讯作者：
Anderson, DJ
DOI：
10.1111/j.1524-4725.1991.tb01597.x
发表时间：
1991-01-01
期刊：
JOURNAL OF DERMATOLOGIC SURGERY AND ONCOLOGY
影响因子：
--
作者：
GERONEMUS, RG;ASHINOFF, R
通讯作者：
ASHINOFF, R