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Related Concept Videos

EPS and iPS Cells in Disease Research01:21

EPS and iPS Cells in Disease Research

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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,...
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Modeling Axonal Phenotypes with Human Pluripotent Stem Cells.

Kyle R Denton1, Chong-Chong Xu1, Xue-Jun Li2,3

  • 1Department of Neuroscience, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT, 06030, USA.

Methods in Molecular Biology (Clifton, N.J.)
|December 19, 2014
PubMed
Summary
This summary is machine-generated.

Human pluripotent stem cells (hPSCs) offer a powerful tool for studying neurodegenerative diseases like hereditary spastic paraplegia (HSP). This study details methods for modeling these conditions using hPSCs to understand axonal defects.

Keywords:
AxonDegenerationMitochondriahESCsiPSCs

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Area of Science:

  • Neuroscience
  • Stem Cell Biology
  • Genetics

Background:

  • Axonal development and degeneration are central to debilitating neurological disorders such as hereditary spastic paraplegia (HSP), amyotrophic lateral sclerosis (ALS), and peripheral neuropathy.
  • Human pluripotent stem cells (hPSCs) are a valuable tool for in vitro modeling of human neuropathologic processes, including axonal defects.

Purpose of the Study:

  • To describe the essential steps for developing human pluripotent stem cell (hPSC)-based models of human diseases.
  • To focus on the application of these methods for modeling the neurodegenerative disorder hereditary spastic paraplegia (HSP).

Main Methods:

  • Generation of induced pluripotent stem cells (iPSCs) from patient samples.
  • Differentiation of iPSCs into specific cell types affected by the disease.
  • Identification and characterization of disease-relevant phenotypes in vitro.

Main Results:

  • Detailed protocols for generating and differentiating hPSCs are presented.
  • Specific methodologies for observing axonal defects in disease models are outlined.
  • The approach is demonstrated with a focus on hereditary spastic paraplegia (HSP).

Conclusions:

  • hPSC-based modeling provides a robust platform for investigating the mechanisms of neurodegenerative diseases.
  • The described methods facilitate the study of axonal defects in conditions like HSP.
  • This approach aids in understanding and potentially developing therapies for debilitating neurological disorders.