Servicio de Neurobiología, Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), 28034 Madrid, Spain.
Stem Cells Int. 2012;2012:135187. doi: 10.1155/2012/135187. Epub 2012 Oct 24.
Cerebellar ataxias include a heterogeneous group of infrequent diseases characterized by lack of motor coordination caused by disturbances in the cerebellum and its associated circuits. Current therapies are based on the use of drugs that correct some of the molecular processes involved in their pathogenesis. Although these treatments yielded promising results, there is not yet an effective therapy for these diseases. Cell replacement strategies using human umbilical cord blood mononuclear cells (HuUCBMCs) have emerged as a promising approach for restoration of function in neurodegenerative diseases. The aim of this work was to investigate the potential therapeutic activity of HuUCBMCs in the 3-acetylpyridine (3-AP) rat model of cerebellar ataxia. Intravenous administered HuUCBMCs reached the cerebellum and brain stem of 3-AP ataxic rats. Grafted cells reduced 3-AP-induced neuronal loss promoted the activation of microglia in the brain stem, and prevented the overexpression of GFAP elicited by 3-AP in the cerebellum. In addition, HuUCBMCs upregulated the expression of proteins that are critical for cell survival, such as phospho-Akt and Bcl-2, in the cerebellum and brain stem of 3-AP ataxic rats. As all these effects were accompanied by a temporal but significant improvement in motor coordination, HuUCBMCs grafts can be considered as an effective cell replacement therapy for cerebellar disorders.
小脑共济失调包括一组罕见疾病,其特征是由于小脑及其相关回路的紊乱导致运动协调缺失。目前的治疗方法基于使用药物来纠正其发病机制中涉及的一些分子过程。尽管这些治疗方法取得了有希望的结果,但对于这些疾病还没有有效的治疗方法。使用人脐带血单核细胞(HuUCBMCs)的细胞替代策略已成为恢复神经退行性疾病功能的有前途的方法。本工作旨在研究 HuUCBMCs 在 3-乙酰吡啶(3-AP)小脑共济失调大鼠模型中的潜在治疗活性。静脉内给予的 HuUCBMCs 到达了 3-AP 共济失调大鼠的小脑和脑干。移植的细胞减少了 3-AP 诱导的神经元丢失,促进了脑干中小胶质细胞的激活,并防止了 3-AP 在小脑中引起的 GFAP 过度表达。此外,HuUCBMCs 上调了小脑和脑干中对细胞存活至关重要的蛋白的表达,如磷酸化 Akt 和 Bcl-2。由于所有这些作用都伴随着运动协调的暂时但显著改善,因此 HuUCBMCs 移植可以被认为是小脑障碍的有效细胞替代治疗方法。