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从患有PHACE综合征的患者中生成人类诱导多能干细胞系。

Generation of human induced pluripotent stem cell lines from patients with PHACE syndrome.

作者信息

Ramchandani Rohin, Cheawsamoot Chanatjit, Torres Giovanni A, Arthur Ataam Jennifer, Siegel Dawn, Karakikes Ioannis

机构信息

Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, CA, USA; Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA, USA.

Department of Dermatology, Stanford University School of Medicine, Stanford, CA, USA.

出版信息

Stem Cell Res. 2025 Sep;87:103802. doi: 10.1016/j.scr.2025.103802. Epub 2025 Aug 9.

Abstract

PHACE syndrome (posterior fossa anomalies, hemangiomas, arterial anomalies, cardiac defects, and eye anomalies) represents a series of multisystem structural birth defects associated with a benign infantile hemangioma. This rare disease presents a variety of symptoms, and its genetic etiology remains unknown. We isolated peripheral mononuclear blood cells (PBMCs) from three patients with PHACE syndrome and generated induced pluripotent stem cell (iPSC) lines using Sendai virus reprogramming. Each line exhibits normal iPSC morphology, high expression of pluripotency factor genes, and trilineage differentiation capability. These lines are valuable for modeling PHACE syndrome in vitro to explore the disease mechanism and screen for potential therapeutics.

摘要

PHACE综合征(后颅窝异常、血管瘤、动脉异常、心脏缺陷和眼部异常)是一系列与良性婴儿血管瘤相关的多系统结构出生缺陷。这种罕见疾病表现出多种症状,其遗传病因仍不清楚。我们从三名PHACE综合征患者中分离出外周血单个核细胞(PBMC),并使用仙台病毒重编程生成诱导多能干细胞(iPSC)系。每个细胞系都表现出正常的iPSC形态、多能性因子基因的高表达以及三系分化能力。这些细胞系对于在体外模拟PHACE综合征以探索疾病机制和筛选潜在治疗方法具有重要价值。

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