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The biophysical characterization of four mutations (T875M, W1204R, R1648H and D1866Y) in SCN1A, the gene encoding the central nervous system (CNS) voltage-gated sodium channel subunit Nav1.1, demonstrated a variety of functional channel defects.
Since SCN5A is the main VGSC expressed in the myocardium and therefore carries the biggest workload, it seems quite reasonable for the cardiomyocyte not to 'waste' RNA or channel protein but to express it as functional channel protein within the cellular membrane.
Monomeric VDAC serves itself as a functional channel [24].
To address the question of whether MβCD altered the number of functional channel complexes in the PM, we recorded voltage-activated whole-cell TRPM8 currents at 20°C.
Acute knockdown of the channel gene decreased the functional channel expression and suppressed the rhythmic firing of the pacemaker neuron, and inhibited breathing activity.
In addition to the various signaling pathways that may modulate the channel activity, ion channel activation also involves subunit assembly/disassembly, trafficking, insertion and endocytosis of functional channel to/from the plasma membrane.
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The ancillary cytoplasmic Ca2+ channel β subunits (CACNB) modulate Ca2+ channel function and are required to enhance the number of functional channels in the plasma membrane.
CNGA1 A3 subunits may form functional channels on their own, while B and A4 subunits serve modulatory functions.
The hydraphiles were intended to be models but they are functional channels.
Hydraulic permeability testing confirmed the presence of functional channels throughout the multilaminate construct.
These functional channel-channel interactions seem to survive isolation and reconstitution in bilayers, where multiple RyRs often display synchronicity named "coupled gating" [11], [12], [13], [14].
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