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Related Concept Videos

G-Protein Gated Ion Channels01:21

G-Protein Gated Ion Channels

GPCRs are primarily responsible for our sense of smell, taste, and vision.  The binding of a sensory stimulus activates GPCR to stimulate effector proteins, many of which are ion channels in the sensory organs. GPCRs modulate the opening and closing of the target ion channels either directly by binding them, or by releasing second messengers that activate these channels. As ions move across the membrane, the membrane potential is altered, which induces an appropriate response.
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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Human Genetics

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Export of Misfolded Proteins out of the ER01:32

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After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
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Related Experiment Video

Updated: Jun 27, 2026

Recapitulation of an Ion Channel IV Curve Using Frequency Components
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Published on: February 8, 2011

Human ether-a-go-go related gene (hERG) K+ channels: function and dysfunction.

Mark J Perrin1, Rajesh N Subbiah, Jamie I Vandenberg

  • 1Victor Chang Cardiac Research Institute, 405 Liverpool Street, Darlinghurst, NSW 2010, Australia.

Progress in Biophysics and Molecular Biology
|November 26, 2008
PubMed
Summary

Mutations in the human Ether-a-go-go Related Gene (hERG) potassium channel cause long QT syndrome. Understanding hERG channel function and drug interactions is key to preventing cardiac arrhythmias and sudden death.

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Last Updated: Jun 27, 2026

Recapitulation of an Ion Channel IV Curve Using Frequency Components
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Published on: February 8, 2011

Isolation and Kv Channel Recordings in Murine Atrial and Ventricular Cardiomyocytes
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Published on: March 12, 2013

Mutagenesis and Functional Analysis of Ion Channels Heterologously Expressed in Mammalian Cells
15:28

Mutagenesis and Functional Analysis of Ion Channels Heterologously Expressed in Mammalian Cells

Published on: October 1, 2010

Area of Science:

  • Cardiovascular Physiology
  • Molecular Cardiology
  • Pharmacology

Background:

  • The human Ether-a-go-go Related Gene (hERG) potassium channel is crucial for cardiac electrical activity and rhythm regulation.
  • hERG channel dysfunction is implicated in both congenital and acquired long QT syndrome, leading to potentially fatal ventricular arrhythmias.
  • The hERG channel is a primary target for drugs that induce acquired long QT syndrome.

Purpose of the Study:

  • To review current knowledge on hERG channel function and its role in cardiac disorders.
  • To summarize advances in understanding hERG gating, trafficking, and drug interactions.
  • To highlight research directions for preventing and treating hERG-associated long QT syndrome.

Main Methods:

  • Literature review of current research on hERG channel.
  • Analysis of structural and functional studies related to hERG gating and drug block.
  • Synthesis of information on hERG channel trafficking and its clinical implications.

Main Results:

  • Detailed understanding of hERG channel's role in cardiac repolarization.
  • Identification of hERG mutations as a cause for congenital long QT syndrome.
  • Recognition of hERG as the molecular target for drug-induced long QT syndrome.

Conclusions:

  • Advances in hERG channel research provide a basis for developing targeted therapies.
  • Further investigation into hERG structure-function relationships is essential for clinical applications.
  • Understanding hERG dysfunction is critical for preventing sudden cardiac death.