Ion permeation pathways are necessary for vital cellular activity such as the establishment of a resting membrane potential and navigation of electrical signals. In body tissues, ions are also known as electrolytes, essential for the electrical activity needed to support muscle contractions and neuron activation.
The physiological importance of ion channel proteins is underlined by a heterogeneous group of disorders, called channelopathies, resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles among with:
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- Diseases of the nervous system (e.g. hyperkalemic and hypokalemic periodic paralysis)
- Diseases of the urinary system (e.g. nephrogenic diabetes insipidus)
- Diseases of the endocrine system (e.g., familial hyperinsulinemic hypoglycemia)
- Diseases of the cardiovascular system (e.g., catecholaminergic polymorphic ventricular tachycardia)
- Diseases of the respiratory system (e.g., cystic fibrosis)
- Diseases of the immune system (e.g. anti-NMDA [N-methyl-D-aspartate] receptor encephalitis)
AMSBIO is offering an extensive range of products involved in ion permeation pathways: