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Related Concept Videos

EPS and iPS Cells in Disease Research01:21

EPS and iPS Cells in Disease Research

Embryonic and induced pluripotent stem cells are excellent models for disease research because of their ability to self-renew and differentiate into most cell types. Somatic cells from a patient are isolated and reprogrammed into induced pluripotent stem cells or iPSCs. These iPSCs are later differentiated into the desired cell type, which mirrors the diseased cell of the patient. In this way, disease models have been created for investigating diseases such as Down syndrome, type I diabetes,...
iPS Cell Differentiation01:22

iPS Cell Differentiation

The ability of induced pluripotent stem cells or iPSCs to differentiate into most body cell types has stimulated repair and regenerative medicine research over the past few decades. iPSC-derived blood cells, hepatocytes, beta islet cells, cardiomyocytes, neurons, and other cell types can repair injuries or regenerate damaged tissue in diseases such as diabetes and neurodegenerative disorders.
Induced Pluripotent Stem Cells01:13

Induced Pluripotent Stem Cells

Stem cells are undifferentiated cells that divide and produce different types of cells. Ordinarily, cells that have differentiated into a specific cell type are post-mitotic—that is, they no longer divide. However, scientists have found a way to reprogram these mature cells so that they “de-differentiate” and return to an unspecialized, proliferative state. These cells are also pluripotent like embryonic stem cells—able to produce all cell types—and are therefore called induced pluripotent stem...
Induced Pluripotent Stem Cells01:06

Induced Pluripotent Stem Cells

Stem cells are undifferentiated cells that divide and produce different cell types. Ordinarily, cells that have differentiated into a specific cell type are terminally differentiated; however, scientists have found a way to reprogram these mature cells so that they dedifferentiate and return to an unspecialized, proliferative state. These cells are pluripotent like embryonic stem cells—able to produce all cell types—and are called induced pluripotent stem cells (iPSCs).
Somatic cells are...
Stem Cell Culture01:17

Stem Cell Culture

Stem cell research aims to find ways to use stem cells to regenerate and repair cellular damage. Over time, most adult cells undergo the wear and tear of aging and lose their ability to divide and repair themselves. Stem cells do not display a particular morphology or function. Adult stem cells, which exist as a small subset of cells in most tissues, keep dividing and can differentiate into a number of specialized cells generally formed by that tissue. These cells enable the body to renew and...
Embryonic Stem Cells00:58

Embryonic Stem Cells

Embryonic stem (ES) cells are undifferentiated pluripotent cells, meaning they can produce any cell type in the body. This gives them tremendous potential in science and medicine since they can generate specific cell types for use in research or to replace body cells lost due to damage or disease.

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Related Experiment Video

Updated: Jul 4, 2026

Generation of Induced Neural Stem Cells from Peripheral Mononuclear Cells and Differentiation Toward Dopaminergic Neuron Precursors for Transplantation Studies
12:13

Generation of Induced Neural Stem Cells from Peripheral Mononuclear Cells and Differentiation Toward Dopaminergic Neuron Precursors for Transplantation Studies

Published on: July 11, 2019

Stem cells in Niemann-Pick disease.

Sun-Jung Kim1, Joon-Suk Park, Kyung-Sun Kang

  • 1Laboratory of Stem Cell and Tumor Biology, College of Veterinary Medicine, Seoul National University, Seoul, Korea.

Disease Markers
|June 6, 2008
PubMed
Summary

Neural stem cell self-renewal is impaired in Niemann-Pick type C1 (NPC1) disease due to NPC1 gene deficiency. This finding offers potential for early NPC disease diagnosis and targeted therapeutics.

Area of Science:

  • Neuroscience
  • Stem Cell Biology
  • Genetics

Background:

  • Neural stem cells (NSCs) possess multipotent and self-renewal capabilities crucial for tissue repair.
  • Loss of NSC self-renewal hinders recovery from cell damage in neurodegenerative diseases.
  • Niemann-Pick type C1 (NPC1) disease is a neurodegenerative disorder caused by mutations in the NPC1 gene, leading to NPC1 protein dysfunction.

Purpose of the Study:

  • To investigate the impact of NPC1 gene deficiency on NSC self-renewal in NPC disease.
  • To identify genes involved in NSC proliferation and differentiation in NPC models.
  • To explore novel diagnostic biomarkers and therapeutic strategies for NPC disease.

Main Methods:

  • Gene profiling of neural stem cells in NPC mice.

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Purification of Prominin-1+ Stem Cells from Postnatal Mouse Cerebellum
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Purification of Prominin-1+ Stem Cells from Postnatal Mouse Cerebellum

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Last Updated: Jul 4, 2026

Generation of Induced Neural Stem Cells from Peripheral Mononuclear Cells and Differentiation Toward Dopaminergic Neuron Precursors for Transplantation Studies
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Purification of Prominin-1+ Stem Cells from Postnatal Mouse Cerebellum

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  • Analysis of stem cell proliferation and differentiation markers.

    • Main Results:

    • NPC1 gene deficiency leads to a loss of self-renewal ability in neural stem cells from NPC disease models.
    • Identification of dysregulated genes associated with stem cell function in NPC mice.

    Conclusions:

    • NPC1 deficiency directly impairs neural stem cell self-renewal, contributing to NPC disease pathology.
    • Understanding NSC behavior in NPC disease can reveal new biomarkers for early diagnosis.
    • Targeted therapeutics focusing on stem cell mechanisms may offer a potential cure for NPC disease.