Related Experiment Video
Updated: Mar 5, 2026

Derivation of Adult Human Fibroblasts and their Direct Conversion into Expandable Neural Progenitor Cells
Published on: July 29, 2015
Abnormal Neural Progenitor Cells Differentiated from Induced Pluripotent Stem Cells Partially Mimicked Development of
Yaqin Li1, Jiqing Cao2, Menglong Chen3
1Department of Neurology, First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China; Department of Neurology, Seventh Affiliated Hospital of Sun Yat-sen University, Shenzhen, Guangdong 518017, P.R. China.
Abstract:
Tuberous sclerosis complex (TSC) is a disease featuring devastating and therapeutically challenging neurological abnormalities. However, there is a lack of specific neural progenitor cell models for TSC. Here, the pathology of TSC was studied using primitive neural stem cells (pNSCs) from a patient presenting a c.1444-2A>C mutation in TSC2. We found that TSC2 pNSCs had higher proliferative activity and increased PAX6 expression compared with those of control pNSCs. Neurons differentiated from TSC2 pNSCs showed enlargement of the soma, perturbed neurite outgrowth, and abnormal connections among cells. TSC2 astrocytes had increased saturation density and higher proliferative activity. Moreover, the activity of the mTOR pathway was enhanced in pNSCs and induced in neurons and astrocytes. Thus, our results suggested that TSC2 heterozygosity caused neurological malformations in pNSCs, indicating that its heterozygosity might be sufficient for the development of neurological abnormalities in patients.
More Related Videos
10:47Efficient Derivation of Human Neuronal Progenitors and Neurons from Pluripotent Human Embryonic Stem Cells with Small Molecule Induction
Published on: October 28, 2011
12:13Generation of Induced Neural Stem Cells from Peripheral Mononuclear Cells and Differentiation Toward Dopaminergic Neuron Precursors for Transplantation Studies
Published on: July 11, 2019
Related Concept Videos
Induced Pluripotent Stem Cells
Induced Pluripotent Stem Cells
Somatic...
iPS Cell Differentiation
EPS and iPS Cells in Disease Research
Stem Cell Culture
Embryonic Stem Cells
ES cells are grown in a culture medium where they can divide indefinitely, creating ES cell lines. Under certain conditions, ES cells can differentiate, either spontaneously into a variety of...