Myotonic dystrophy type 1 management and therapeutics

CA Smith, L Gutmann - Current treatment options in neurology, 2016 - Springer
Opinion statement Myotonic dystrophy (DM1) is the most common form of adult muscular
dystrophy. It is a multisystem disorder with a complex pathophysiology. Although inheritance …

Small conductance Ca2+-activated K+ channels in the plasma membrane, mitochondria and the ER: Pharmacology and implications in neuronal diseases

B Honrath, IE Krabbendam, C Culmsee… - Neurochemistry …, 2017 - Elsevier
Abstract Ca 2+-activated K+(K Ca) channels regulate after-hyperpolarization in many types
of neurons in the central and peripheral nervous system. Small conductance Ca 2+ …

Depressed synaptic transmission and reduced vesicle release sites in Huntington's disease neuromuscular junctions

A Khedraki, EJ Reed, SH Romer, Q Wang… - Journal of …, 2017 - Soc Neuroscience
Huntington's disease (HD) is a progressive and fatal degenerative disorder that results in
debilitating cognitive and motor dysfunction. Most HD studies have focused on degeneration …

Emerging Role of the Calcium-Activated, Small Conductance, SK3 K+ Channel in Distal Tubule Function: Regulation by TRPV4

J Berrout, M Mamenko, OL Zaika, L Chen, W Zang… - PloS one, 2014 - journals.plos.org
The Ca2+-activated, maxi-K (BK) K+ channel, with low Ca2+-binding affinity, is expressed in
the distal tubule of the nephron and contributes to flow-dependent K+ secretion. In the …

Chronic muscle stimulation improves muscle function and reverts the abnormal surface EMG pattern in myotonic dystrophy: a pilot study

C Chisari, F Bertolucci, S Dalise, B Rossi - Journal of Neuroengineering …, 2013 - Springer
Abstract Background To date, in Myotonic Dystrophy type 1 (DM1) the rehabilitative
interventions have always been aimed at muscle strengthening, increasing of fatigue …

Role of SK channel activation in determining the action potential configuration in freshly isolated human atrial myocytes from the SKArF study

YA Shamsaldeen, L Culliford, M Clout… - Biochemical and …, 2019 - Elsevier
Inhibition of SK channel function is being pursued in animal models as a possible
therapeutic approach to treat atrial fibrillation (AF). However, the pharmacology of SK …

Increased contribution of KCa channels to muscle contraction induced vascular and blood flow responses in sedentary and exercise trained ZFDM rats

T Sonobe, H Tsuchimochi, H Maeda… - The Journal of …, 2022 - Wiley Online Library
In resistance arteries, endothelium‐dependent hyperpolarization (EDH)‐mediated
vasodilatation is depressed in diabetes. We hypothesized that downregulation of KCa …

[图书][B] Tissue-specific cell signaling

JV Silva, MJ Freitas, M Fardilha - 2020 - Springer
Cells have evolved to sense external chemical and physical cues, transduce the signals
intracellularly through molecular cascades, and generate an appropriate response. The …

Multifocal Noninvasive Magnetic Stimulation of the Primary Motor Cortex in Type 1 Myotonic Dystrophy–A Proof of Concept Pilot Study

E Greene, J Thonhoff, BS John… - Journal of …, 2021 - content.iospress.com
Background: Repeated neuromuscular electrical stimulation in type 1 Myotonic Dystrophy
(DM1) has previously been shown to cause an increase in strength and a decrease in …

The signaling pathways involved in the regulation of skeletal muscle plasticity

A Moreira-Pais, F Amado, R Vitorino… - Tissue-Specific Cell …, 2020 - Springer
Signal transduction is essential for skeletal muscle functionality, a highly plastic tissue able
to respond to mechanical and chemical stimuli by altering its metabolism, size and myogenic …