4-phenylbutyric acid re-trafficking hERG/G572R channel protein by modulating the endoplasmic reticulum stress-associated chaperones and endoplasmic reticulum-associated degradation gene
CONCLUSIONS: 4-PBA corrects hERG channel transport defects by inhibiting excessive ERS and the endoplasmic reticulum-associated degradation (ERAD)-related gene E3 ubiquitin ligase HRD1. Additionally, 4-PBA improved WT/G572R channel current. 4-PBA is expected to be developed as a new treatment method for LQT2.PMID:37691654 | PMC:PMC10482650 | DOI:10.21037/jtd-23-1252 (Source: Journal of Thoracic Disease)
Source: Journal of Thoracic Disease - September 11, 2023 Category: Respiratory Medicine Authors: Wen Tang Dihui Cai Yin Fu Zequn Zheng Xiaoyan Huang Rami N Khouzam Yongfei Song Jiangfang Lian Source Type: research