The role of the Notch signaling pathway in neural development has been well established over many years. More recent studies, however, have demonstrated that Notch continues to be expressed and active throughout adulthood in many areas of the central nervous system. Notch signals have been implicated in adult neurogenesis, memory formation, and synaptic plasticity in the adult organism, as well as linked to acute brain trauma and chronic neurodegenerative conditions. NOTCH3 mutations are responsible for the most common form of hereditary stroke, the progressive disorder cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Notch has also been associated with several progressive neurodegenerative diseases, including Alzheimer's disease, multiple sclerosis, and amyotrophic lateral sclerosis. Although numerous studies link Notch activity with CNS homeostasis and neurodegenerative diseases, the data thus far are primarily correlative, rather than functional. Nevertheless, the evidence for Notch pathway activity in specific neural cellular contexts is strong, and certainly intriguing, and points to the possibility that the pathway carries therapeutic promise.

中文翻译：

---

中枢神经系统稳态和神经退行性病变中的Notch通路。

多年来，Notch信号通路在神经发育中的作用已被很好地确立。然而，最近的研究表明，Notch在成年后在中枢神经系统的许多区域继续表达并活跃。Notch信号与成人生物体内的成人神经发生，记忆形成和突触可塑性有关，并且与急性脑外伤和慢性神经退行性疾病有关。缺口3突变是遗传性中风的最常见形式，是进行性疾病，常染色体显性遗传性脑病，伴有皮下梗塞和白质脑病。Notch还与几种进行性神经退行性疾病有关，包括阿尔茨海默氏病，多发性硬化症和肌萎缩性侧索硬化症。尽管许多研究将Notch活性与CNS稳态和神经退行性疾病联系起来，但到目前为止，这些数据主要是相关的，而不是功能性的。尽管如此，Notch途径在特定神经细胞环境中活动的证据是有力的，并且确实很有趣，并且指出了该途径具有治疗前景的可能性。