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异性错误的CSF1R变体阻碍了体外CD34+衍生型树突细胞的产生,但体内树突细胞的发育并未发生

Shanice Beerepoot¹, Nicole I Wolf², Marjo S van der Knaap³

¹Department of Child Neurology, Amsterdam Leukodystrophy Center, Emma Children's Hospital, Amsterdam University Medical Center, Amsterdam, The Netherlands; Center for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.

Molecular immunology

|August 25, 2024

概括

殖民地刺激因子1受体 (CSF1R) 中的致病变体在CSF1R相关疾病患者的干细胞中损害了体外树突细胞 (DC) 的发育. 然而,直流的发展似乎在体内正常,这表明有补偿机制.

科学领域:

免疫学免疫学免疫学
神经科学是一个神经科学.
遗传学是一个遗传学.

背景情况:

殖民地刺激因子1受体 (CSF1R) 信号传递对髓状细胞发育至关重要,包括单细胞和微质细胞.
由致病性CSF1R变异引起的CSF1R相关疾病 (CSF1R-RD) 是一种神经疾病,其特征是小质减少和单细胞功能改变.
CSF1R变异对人类树突细胞 (DC) 谱系发展的影响尚不清楚.

研究的目的:

研究与CSF1R相关疾病的患者中异性致病性CSF1R变异对人类DC血统的影响.
为了比较体外DC分化潜力从CD34+干细胞和CD14+单细胞的患者与健康个体.

主要方法:

从四名患有CSF1R-RD和健康对照的非相关患者中分离出了外围血液CD34+干细胞和CD14+单细胞.
细胞在体外培养以分化为传统的DC和单细胞衍生的DC.
使用流细胞计分析DC差异化和子群群体.

主要成果:

来自CSF1R-RD患者的单细胞可以在体外分化为单细胞衍生的DC.
在患者体内,CD34+干细胞分化成常规DCs受损.
所有自然存在的DC子集都存在于患者的外周血液中.

结论:

在CSF1R-RD患者的干细胞中体外受损的DC发育不一定反映在体内DC发育.
在体内可以补偿CSF1R信号,允许正常的DC发展,尽管有致病变体.
需要进一步的研究来阐明CSF1R信号的补偿机制.

关键词:

成人发病的白细胞大脑病变 (leukoencephalopathy) 带有轴突球体和有色素的质细胞.这种基因是CSF1R.殖民地刺激因子1受体受体树突细胞的树突细胞.单细胞 (Monocytes) 是一种单细胞.

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