EasySep™小鼠TIL（CD45）正选试剂盒
产品号 #60053_C
小鼠抗人、小鼠、大鼠少突胶质细胞标记物O4的单克隆IgM抗体
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总览
81抗体克隆与O4抗原反应，这是一种硫酸半乳糖脑苷（硫脂）。O 抗原主要表达于中枢神经系统中正在分化和成熟的少突胶质细胞的髓鞘质膜外侧叶，以及周围神经系统的雪旺细胞上。O4是在出生后发育过程中形成的，是 I 型和 II 型少突胶质细胞胞体和突起的标志物。O4在前少突胶质细胞中表达，但在O-2A祖细胞中不表达。O4的异常表达与多种疾病有关，包括神经纤维瘤病和精神分裂症。
亚型
一抗
靶抗原
Oligodendrocyte Marker O4
别名
3-O-磺基半乳糖苷神经酰胺，SM4，硫苷脂
活性物种
Chicken，人，小鼠，大鼠
偶联
未偶联的
宿主物种
小鼠
细胞类型
神经细胞，PSC衍生，神经干/祖细胞
种属
人，小鼠，其它细胞系，大鼠
应用
流式细胞术，免疫细胞化学，免疫荧光，免疫组化
研究领域
神经科学
克隆
81
同种型
IgM
产品说明书及文档
请在《产品说明书》中查找相关支持信息和使用说明，或浏览下方更多实验方案。
应用领域
本产品专为以下研究领域设计，适用于工作流程中的高亮阶段。探索这些工作流程，了解更多我们为各研究领域提供的其他配套产品。
相关材料与文献
技术资料 (2)
文献 (1)
Modeling neuronal defects associated with a lysosomal disorder using patient-derived induced pluripotent stem cells. Human Molecular Genetics 2011 SEP
Abstract
By providing access to affected neurons, human induced pluripotent stem cells (iPSc) offer a unique opportunity to model human neurodegenerative diseases. We generated human iPSc from the skin fibroblasts of children with mucopolysaccharidosis type IIIB. In this fatal lysosomal storage disease, defective α-N-acetylglucosaminidase interrupts the degradation of heparan sulfate (HS) proteoglycans and induces cell disorders predominating in the central nervous system, causing relentless progression toward severe mental retardation. Partially digested proteoglycans, which affect fibroblast growth factor signaling, accumulated in patient cells. They impaired isolation of emerging iPSc unless exogenous supply of the missing enzyme cleared storage and restored cell proliferation. After several passages, patient iPSc starved of an exogenous enzyme continued to proliferate in the presence of fibroblast growth factor despite HS accumulation. Survival and neural differentiation of patient iPSc were comparable with unaffected controls. Whereas cell pathology was modest in floating neurosphere cultures, undifferentiated patient iPSc and their neuronal progeny expressed cell disorders consisting of storage vesicles and severe disorganization of Golgi ribbons associated with modified expression of the Golgi matrix protein GM130. Gene expression profiling in neural stem cells pointed to alterations of extracellular matrix constituents and cell-matrix interactions, whereas genes associated with lysosome or Golgi apparatus functions were downregulated. Taken together, these results suggest defective responses of patient undifferentiated stem cells and neurons to environmental cues, which possibly affect Golgi organization, cell migration and neuritogenesis. This could have potential consequences on post-natal neurological development, once HS proteoglycan accumulation becomes prominent in the affected child brain.
更多信息
| 种属 | Human, Mouse, Other, Rat |
|---|---|
| 克隆 | 81 |
| Alternative Names | 3-O-sulfogalactosylceramide, SM4, Sulfatide |
| 同种型/isotype | IgM |
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