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Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry

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Published on: September 28, 2016

Channelopathies: a review.

Genevieve Bernard¹, Michael I Shevell

¹Department of Neurology/Neurosurgery, McGill University, Montreal Children's Hospital-McGill University Health Center, Montreal, Quebec, Canada.

Pediatric Neurology

|January 22, 2008

Summary

Channelopathies are rare neurologic disorders caused by genetic ion channel defects. This review categorizes them by clinical symptoms like weakness and epilepsy, aiding diagnosis.

Area of Science:

Neurology
Genetics
Molecular Biology

Background:

Channelopathies represent a growing category of neurologic disorders.
These conditions stem from genetically determined ion channel dysfunctions.
Significant genetic and phenotypic diversity complicates their understanding.

Purpose of the Study:

To systematically review and categorize channelopathies.
To correlate clinical manifestations with molecular underpinnings.
To aid clinicians in diagnosis and genetic testing.

Main Methods:

Systematic literature review.
Categorization based on predominant clinical features (myotonia, weakness, migraine, ataxia, epilepsy, movement disorders).
Analysis of genetic and phenotypic overlaps.

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Voltage-Dependent Potassium Current Recording on H9c2 Cardiomyocytes via the Whole-Cell Patch-Clamp Technique

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Main Results:

Categorization of channelopathies by clinical presentation.
Identification of molecular bases for various syndromes.
Highlighting of genetic and phenotypic overlaps between disorders.

Conclusions:

Categorizing channelopathies by clinical manifestation aids understanding.
Knowledge of molecular basis is crucial for diagnosis.
This review assists clinicians in diagnostic acumen and targeted genetic testing.