Astroglia are the most abundant glia cell in the central nervous system, playing essential roles in maintaining homeostasis. Key functions of astroglia include, but are not limited to, neurotransmitter recycling, ion buffering, immune modulation, neurotrophin secretion, neuronal synaptogenesis and elimination, and blood–brain barrier maintenance. In neurological diseases, it is well appreciated that astroglia play crucial roles in the disease pathogenesis. In amyotrophic lateral sclerosis (ALS), a motor neuron degenerative disease, astroglia in the spinal cord and cortex downregulate essential transporters, among other proteins, that exacerbate disease progression. Spinal cord astroglia undergo dramatic transcriptome dysregulation. However, in the cortex, it has not been well studied what effects glia, especially astroglia, have on upper motor neurons in the pathology of ALS. To begin to shed light on the involvement and dysregulation that astroglia undergo in ALS, we isolated pure grey-matter cortical astroglia and subjected them to microarray analysis. We uncovered a vast number of genes that show dysregulation at end-stage in the ALS mouse model, G93A SOD1. Many of these genes play essential roles in ion homeostasis and the Wnt-signaling pathway. Several of these dysregulated genes are common in ALS spinal cord astroglia, while many of them are unique. This database serves as an approach for understanding the significance of dysfunctional genes and pathways in cortical astroglia in the context of motor neuron disease, as well as determining regional astroglia heterogeneity, and providing insight into ALS pathogenesis.

星形胶质细胞是中枢神经系统中最丰富的神经胶质细胞，在维持体内平衡中发挥着重要作用。星形胶质细胞的关键功能包括但不限于神经递质回收、离子缓冲、免疫调节、神经营养蛋白分泌、神经元突触发生和消除以及血脑屏障维持。在神经系统疾病中，人们普遍认识到星形胶质细胞在疾病发病机制中发挥着至关重要的作用。在肌萎缩侧索硬化症（ALS）这种运动神经元退行性疾病中，脊髓和皮质中的星形胶质细胞会下调必需转运蛋白以及其他蛋白质，从而加剧疾病进展。脊髓星形胶质细胞经历剧烈的转录组失调。然而，在皮质中，神经胶质细胞（尤其是星形胶质细胞）对 ALS 病理学中上运动神经元的影响尚未得到充分研究。为了开始阐明星形胶质细胞在 ALS 中所经历的参与和失调，我们分离了纯灰质皮质星形胶质细胞，并对它们进行了微阵列分析。我们在 ALS 小鼠模型G93A SOD1中发现了大量在终末期表现出失调的基因。其中许多基因在离子稳态和 Wnt 信号通路中发挥着重要作用。其中一些失调基因在 ALS 脊髓星形胶质细胞中很常见，但其中许多是独特的。该数据库可作为一种方法，用于了解运动神经元疾病背景下皮质星形胶质细胞功能失调的基因和通路的重要性，以及确定区域星形胶质细胞的异质性，并提供对 ALS 发病机制的深入了解。