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논문분류	춘계학술대회 초록집
제목	Therapeutic Potential of Propylparaben in Modulating Pathophysiological Features of Fabry Nephropathy
저자	Sungjin Chung
출판정보	2025; 2025(1):
키워드	Fabry disease, Kidney disease, Inflammation, Fibrosis, Treatment
초록	Fabry disease is a rare X-linked lysosomal storage disorder caused by mutation in the GLA gene, resulting in deficient α-galactosidase A enzyme activity, leading to progressive multi-organ damage, including kidney failure. Despite the widespread use of enzyme replacement therapy (ERT), some patients experience disease progression. This study evaluated the therapeutic potential of propylparaben (PP) in a Fabry disease model, focusing on renal fibrosis, oxidative stress, inflammation, and urine albumin excretion. Histological changes, inflammatory markers, oxidative stress markers, and lipid peroxidation in the kidneys were assessed in wild-type control mice, untreated Fabry disease mice, and Fabry disease mice treated with PP. Fabry disease mice exhibited increased renal interstitial fibrosis compared to wild-type controls, as evidenced by trichrome and Sirius red staining, but PP treatment significantly reduced fibrosis in Fabry disease mice. Elevated renal mRNA levels of pro-inflammatory markers such as IL-1β and IL-6 in Fabry disease were downregulated by PP treatment. In PP-treated Fabry disease mice, renal HO-1 protein levels were significantly increased and 4-HNE expression was reduced. Additionally, Fabry disease mice showed elevated albuminuria levels, which were significantly reduced following PP treatment. This study demonstrates that PP mitigates renal inflammation, fibrosis, oxidative stress, and albuminuria in a Fabry disease model. These findings suggest that PP holds promise as a potential therapeutic option, warranting further investigation in clinical settings.
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