Channelopathies of skeletal muscle excitability

SC Cannon - Comprehensive Physiology, 2015‏ - pmc.ncbi.nlm.nih.gov
Familial disorders of skeletal muscle excitability were initially described early in the last
century and are now known to be caused by mutations of voltage-gated ion channels. The …

Therapeutic approaches to genetic ion channelopathies and perspectives in drug discovery

P Imbrici, A Liantonio, GM Camerino… - Frontiers in …, 2016‏ - frontiersin.org
In the human genome more than 400 genes encode ion channels, which are
transmembrane proteins mediating ion fluxes across membranes. Being expressed in all …

Mechanism of thyrotoxic periodic paralysis

SH Lin, CL Huang - Journal of the American Society of …, 2012‏ - journals.lww.com
The pathogenesis of thyrotoxic periodic paralysis has long been thought related to
increased Na+–K+ ATPase activity stimulated by thyroid hormone and/or hyperadrenergic …

Inwardly rectifying potassium channel Kir2. 1 and its “Kir-ious” regulation by protein trafficking and roles in development and disease

NA Hager, CK McAtee, MA Lesko… - Frontiers in Cell and …, 2022‏ - frontiersin.org
Potassium (K+) homeostasis is tightly regulated for optimal cell and organismal health.
Failure to control potassium balance results in disease, including cardiac arrythmias and …

A 10-year analysis of thyrotoxic periodic paralysis in 135 patients: focus on symptomatology and precipitants

CC Chang, CJ Cheng, CC Sung… - European journal of …, 2013‏ - academic.oup.com
Background A comprehensive analysis has not been performed on patients with thyrotoxic
periodic paralysis (TPP) characterized by acute hypokalemia and paralysis in the setting of …

Extracellular potassium homeostasis: insights from hypokalemic periodic paralysis

CJ Cheng, E Kuo, CL Huang - Seminars in nephrology, 2013‏ - Elsevier
Extracellular potassium makes up only about 2% of the total body's potassium store. The
majority of the body potassium is distributed in the intracellular space, of which about 80% is …

[Retracted] Thyrotoxic Periodic Paralysis: Clinical Challenges

A Vijayakumar, G Ashwath… - Journal of thyroid …, 2014‏ - Wiley Online Library
Thyrotoxic periodic paralysis (TPP), a disorder most commonly seen in Asian men, is
characterized by abrupt onset of hypokalemia and paralysis. The condition primarily affects …

[HTML][HTML] Ion channel gene mutations causing skeletal muscle disorders: pathomechanisms and opportunities for therapy

L Maggi, S Bonanno, C Altamura, JF Desaphy - Cells, 2021‏ - mdpi.com
Skeletal muscle ion channelopathies (SMICs) are a large heterogeneous group of rare
genetic disorders caused by mutations in genes encoding ion channel subunits in the …

Probing ion channel functional architecture and domain recombination compatibility by massively parallel domain insertion profiling

W Coyote-Maestas, D Nedrud, A Suma, Y He… - Nature …, 2021‏ - nature.com
Protein domains are the basic units of protein structure and function. Comparative analysis
of genomes and proteomes showed that domain recombination is a main driver of …

Periodic paralysis

D Fialho, RC Griggs, E Matthews - Handbook of clinical neurology, 2018‏ - Elsevier
The periodic paralyses are a group of skeletal muscle channelopathies characterizeed by
intermittent attacks of muscle weakness often associated with altered serum potassium …