Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

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,...
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...
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...
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.

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A case of CD36 deficiency with multiple white matter lesions.

BMC neurology·2026
Same author

A novel selective stabilizer of the ryanodine receptor 2 prevents stress-induced ventricular arrhythmias without impairing cardiac function.

British journal of pharmacology·2026
Same author

Generation of functional mesothelial cells from human iPSCs that restore peritoneal integrity in experimental peritoneal injury.

Stem cell research & therapy·2026
Same author

Nihon yakurigaku zasshi. Folia pharmacologica Japonica·2026
Same author

Cardiac characteristics of Fabry disease from baseline enrolment data in a nationwide prospective Japanese registry.

International journal of cardiology·2025
Same author

Specific induction of right ventricular-like cardiomyocytes from human pluripotent stem cells.

Stem cell research & therapy·2025

Related Experiment Video

Updated: May 21, 2026

Derivation and Characterization of a Transgene-free Human Induced Pluripotent Stem Cell Line and Conversion into Defined Clinical-grade Conditions
10:48

Derivation and Characterization of a Transgene-free Human Induced Pluripotent Stem Cell Line and Conversion into Defined Clinical-grade Conditions

Published on: November 26, 2014

Disease characterization using LQTS-specific induced pluripotent stem cells.

Toru Egashira1, Shinsuke Yuasa, Tomoyuki Suzuki

  • 1Department of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan.

Cardiovascular Research
|June 29, 2012
PubMed
Summary

Induced pluripotent stem cells (iPSCs) effectively model Long QT syndrome (LQTS) by characterizing novel mutations and predicting drug responses, paving the way for personalized cardiac therapies.

More Related Videos

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells
08:52

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells

Published on: June 13, 2018

Generation of 3D Whole Lung Organoids from Induced Pluripotent Stem Cells for Modeling Lung Developmental Biology and Disease
09:45

Generation of 3D Whole Lung Organoids from Induced Pluripotent Stem Cells for Modeling Lung Developmental Biology and Disease

Published on: April 12, 2021

Related Experiment Videos

Last Updated: May 21, 2026

Derivation and Characterization of a Transgene-free Human Induced Pluripotent Stem Cell Line and Conversion into Defined Clinical-grade Conditions
10:48

Derivation and Characterization of a Transgene-free Human Induced Pluripotent Stem Cell Line and Conversion into Defined Clinical-grade Conditions

Published on: November 26, 2014

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells
08:52

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells

Published on: June 13, 2018

Generation of 3D Whole Lung Organoids from Induced Pluripotent Stem Cells for Modeling Lung Developmental Biology and Disease
09:45

Generation of 3D Whole Lung Organoids from Induced Pluripotent Stem Cells for Modeling Lung Developmental Biology and Disease

Published on: April 12, 2021

Area of Science:

  • Cardiovascular Research
  • Stem Cell Biology
  • Genetics

Background:

  • Long QT syndrome (LQTS) is a life-threatening inherited disorder.
  • Sporadic LQTS cases with novel mutations pose diagnostic challenges.
  • Precise characterization is crucial for developing targeted therapies.

Observation:

  • Somatic cells from an LQTS patient and controls were reprogrammed into induced pluripotent stem cells (iPSCs).
  • iPSCs were differentiated into cardiomyocytes using embryoid body (EB) formation.
  • Electrophysiological analysis of LQTS-iPSC-derived EBs revealed prolonged field potential duration (FPD).

Findings:

  • IKr and IKs channel blockers differentially affected LQTS-iPSC-derived EBs compared to controls.
  • E4031 (IKr blocker) induced severe arrhythmias specifically in LQTS-iPSC-derived EBs.
  • Chromanol 293B (IKs blocker) prolonged FPD in control EBs but not LQTS-iPSC-derived EBs, indicating IKs disturbance.
  • Patch-clamp and immunostaining confirmed a dominant-negative effect of the 1893delC mutation in IKs channels due to trafficking deficiency.

Implications:

  • iPSCs are valuable tools for characterizing LQTS disease mechanisms.
  • iPSC-derived cardiomyocytes enable prediction of drug responses in LQTS patients with novel mutations.
  • This approach supports the advancement of personalized medicine for cardiac ion channel disorders.