技术资料

Baarine M et al. (NOV 2015) PLoS ONE 10 11 e0143238

Functional characterization of IPSC-derived brain cells as a model for X-linked adrenoleukodystrophy

X-ALD is an inherited neurodegenerative disorder where mutations in the ABCD1 gene result in clinically diverse phenotypes: the fatal disorder of cerebral childhood ALD (cALD) or a milder disorder of adrenomyeloneuropathy (AMN). The various models used to study the pathobiology of X-ALD disease lack the appropriate presentation for different phenotypes of cALD vs AMN. This study demonstrates that induced pluripotent stem cells (IPSC) derived brain cells astrocytes (Ast),neurons and oligodendrocytes (OLs) express morphological and functional activities of the respective brain cell types. The excessive accumulation of saturated VLCFA,a hallmark" of X-ALD� View Publication

产品号#：

05850

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85857

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27845

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产品名：

mTeSR™1

mTeSR™1

STEMdiff™ 神经诱导培养基

STEMdiff™ 神经诱导培养基

STEMdiff™SMADi神经诱导试剂盒

STEMdiff™SMADi神经诱导试剂盒，2套
Fuller HR et al. (JAN 2015) Frontiers in cellular neuroscience 9 January 506

Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Have Reduced Expression of Proteins Important in Neuronal Development.

Spinal muscular atrophy (SMA) is an inherited neuromuscular disease primarily characterized by degeneration of spinal motor neurons,and caused by reduced levels of the SMN protein. Previous studies to understand the proteomic consequences of reduced SMN have mostly utilized patient fibroblasts and animal models. We have derived human motor neurons from type I SMA and healthy controls by creating their induced pluripotent stem cells (iPSCs). Quantitative mass spectrometry of these cells revealed increased expression of 63 proteins in control motor neurons compared to respective fibroblasts,whereas 30 proteins were increased in SMA motor neurons vs. their fibroblasts. Notably,UBA1 was significantly decreased in SMA motor neurons,supporting evidence for ubiquitin pathway defects. Subcellular distribution of UBA1 was predominantly cytoplasmic in SMA motor neurons in contrast to nuclear in control motor neurons; suggestive of neurodevelopmental abnormalities. Many of the proteins that were decreased in SMA motor neurons,including beta III-tubulin and UCHL1,were associated with neurodevelopment and differentiation. These neuron-specific consequences of SMN depletion were not evident in fibroblasts,highlighting the importance of iPSC technology. The proteomic profiles identified here provide a useful resource to explore the molecular consequences of reduced SMN in motor neurons,and for the identification of novel biomarker and therapeutic targets for SMA. View Publication

产品号#：

05832

05850

05857

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产品名：

STEMdiff™ 神经花环选择试剂

mTeSR™1

mTeSR™1
产品手册
STEMCELL 神经科学研究工具

品牌：

BrainPhys
产品手册
Modeling Neuroinflammation with hPSC-Derived Cells

品牌：

STEMdiff
研究综述
The Predictive Power of Organoid-Based New Approach Methodologies in Drug Discovery

细胞类型：

上皮细胞，多能干细胞，肠道细胞，肾细胞，胰腺细胞，PSC衍生上皮细胞，PSC衍生肝细胞，呼吸道细胞
实验方案
How to Generate AssemBloids™ from hPSC-Derived Dorsal and Ventral Forebrain Organoid Co-Cultures

研究方向：

疾病建模，神经科学，类器官
31:35:00

视频
Neuronal Phenotyping of an iPS Cell Model of Rett Syndrome
53:16:00

视频
Modeling Genetic Epilepsies with Induced Pluripotent Stem Cells
47:18:00

视频
Madeline Lancaster on Brain Organoids: Modeling Human Brain Development in a Dish
53:22:00

视频
Using hiPSCs to Study Genetic Variants Linked to Schizophrenia
17:30

视频
Nature Research Round Table: Human Pluripotent Stem Cell Lines as Disease Models
科学海报
A Reliable, Efficient, and Matrix-Free Method to Generate Midbrain Organoids from Human Pluripotent Stem Cells

Conference：

CSHL 3D Brain 2022