
Mutation-specific peripheral and ER quality control of hERG …
2019年4月15日 · Here, we investigate the cellular processing of mildly-misfolded Per-Arnt-Sim (PAS)-domain mutant hERGs, which display incomplete ER-retention and PM-expression …
Rescue of expression and function of long QT syndrome-causing mutant …
2024年8月1日 · In the present study, we propose that certain mutant hERG channels, such as G601S, may display enhanced instability of the channel, causing it to behave like WT hERG in …
Mechanisms of pharmacological rescue of trafficking defective hERG ...
Mutations in the human ether-a-go-go-related gene (hERG) cause long QT syndrome type 2 (LQT2) (2). hERG encodes the pore-forming subunit of the rapidly activating delayed rectifier …
Reciprocal Control of hERG Stability by Hsp70 and Hsc70 With ...
2010年12月23日 · We investigated whether Hsc70 counteracts Hsp70 in the control of wild-type and mutant hERG stability. Coexpression of Hsp70 with hERG in HEK293 cells suppressed …
Pharmacologic Approach to Defective Protein Trafficking in …
2013年6月19日 · It is reported that PD-118057 and thapsigargin can rescue LQT2 without hERG channel blockade, but the precise mechanism of action is unknown. Furthermore, the effect of …
Restoration of mutant hERG stability by inhibition of HDAC6
2018年2月1日 · Three acetylated lysines in hERG have been identified. HDAC6 inhibition increases endogenous ERG and IKr in HL-1 mouse cardiomyocytes. The human ether-a-go …
Mutant MiRP1 subunits modulate HERG K - PubMed Central …
Mutations in KCNE2, which encodes the minK-related protein 1 (MiRP1), are associated with an increased risk of arrhythmias; however, the underlying mechanisms are unknown. MiRP1 is …
Frontiers | When HERG-caused LQT2 encounters antisense …
6 天之前 · For diseases caused by specific mutations, such as the exon 6 mutations in HERG associated with LQT2, the ideal ASO would specifically target transcripts from the mutant …
Rescue of expression and function of long QT syndrome-causing mutant …
2024年7月2日 · Reduced temperature culture rescues the membrane expression and function of G601S hERG mutant. It has been established that hERG proteins from hERG-expressing …
An NMR study of the N-terminal domain of wild-type hERG and a …
The human Ether-à-go-go Related Gene (hERG) potassium channel plays an important role in the heart by controlling the rapid delayed rectifier current. The N-terminal 135 residues (NTD) …
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