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Related Concept Videos

Somatic to iPS Cell Reprogramming01:29

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Reprogramming alters the gene expression in somatic cells, transforming them into induced pluripotent stem (iPS) cells over several generations. Scientists can reprogram cells by introducing genes for four transcription factors—Oct4, Sox2, Klf4, and c-Myc (OSKM) by viral or non-viral methods. These factors are also known as Yamanaka factors after Shinya Yamanaka, who first generated iPS cells using mouse skin cells. Yamanaka was awarded the Nobel Prize in Physiology or Medicine in 2012...
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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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Cell Reprogramming to Model Huntington's Disease: A Comprehensive Review.

Ruth Monk1, Bronwen Connor1

  • 1Centre for Brain Research, Department of Pharmacology and Clinical Pharmacology, Faculty of Medical and Health Sciences, School of Medical Science, University of Auckland, Auckland 1023, New Zealand.

Cells
|July 2, 2021
PubMed
Summary

Cell reprogramming generates patient-specific neurons, offering new ways to study Huntington's disease (HD) mechanisms and test drugs. This technology holds promise for developing future Huntington's disease therapies.

Keywords:
Huntington’s diseasecell reprogrammingdirect cell reprogrammingdisease modellingpluripotent stem cellsstriatal differentiation

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is a fatal neurodegenerative disorder caused by a genetic mutation leading to mutant Huntingtin protein (mHTT).
  • Selective loss of medium spiny neurons (MSNs) in the striatum is a hallmark of HD pathogenesis.
  • Current understanding of HD mechanisms is limited by the lack of access to live patient neurons.

Purpose of the Study:

  • To review the application of cell reprogramming technologies for modeling Huntington's disease.
  • To discuss advancements in cell reprogramming relevant to HD research.
  • To highlight the potential of cell reprogramming for therapeutic development in HD.

Main Methods:

  • Utilizing cell reprogramming to generate patient-derived neurons in vitro.
  • Analyzing the applications of these reprogrammed neurons for disease modeling.
  • Reviewing recent technological advancements in cell reprogramming.

Main Results:

  • Cell reprogramming enables the creation of live, disease-affected neurons from HD patients.
  • These neurons are valuable for studying HD pathogenesis and identifying therapeutic targets.
  • Reprogrammed cells offer potential for high-throughput drug screening and cell replacement therapy.

Conclusions:

  • Cell reprogramming is a powerful tool for in vitro modeling of Huntington's disease.
  • This technology facilitates the investigation of selective MSN degeneration in HD.
  • Advancements in cell reprogramming offer significant potential for future HD therapeutic strategies.