Ketogenic Diet and Progression of Kidney Disease in Animal Models of Nephropathic Cystinosis.

IF 10.3 1区 医学 Q1 UROLOGY & NEPHROLOGY Journal of The American Society of Nephrology Pub Date : 2024-07-12 DOI:10.1681/ASN.0000000000000439
Francesco Bellomo, Sara Pugliese, Sara Cairoli, Patrick Krohn, Cristiano De Stefanis, Roberto Raso, Laura Rita Rega, Anna Taranta, Ester De Leo, Andrea Ciolfi, Nicolò Cicolani, Stefania Petrini, Alessandro Luciani, Bianca Maria Goffredo, Ottavia Porzio, Olivier Devuyst, Carlo Dionisi-Vici, Francesco Emma
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生酮饮食与肾病性膀胱炎动物模型中肾脏疾病的进展。
背景:肾病性胱氨酸病是一种罕见的遗传性溶酶体贮积症,由编码溶酶体胱氨酸/H+合酶的胱苷蛋白的 CTNS 基因突变引起。从肾脏的角度来看,患者会出现早发性肾范可尼综合征和进行性慢性肾病。目前使用半胱胺的疗法可以延缓但不能防止肾衰竭,而且副作用很大,限制了患者的坚持治疗,降低了患者的生活质量:我们从生化和组织学角度测试了生酮饮食对两种肾病性胱氨酸沉积症动物模型肾脏疾病的影响:结果:当 Ctns-/- 小鼠从 3 个月大到 12 个月大期间饲喂生酮饮食时,我们观察到小鼠几乎完全缓解了范可尼综合征,包括低分子量蛋白尿、糖尿和多尿。与野生型动物相比,用标准饮食喂养的胱氨酸瘤小鼠在 12 个月大时的血清尿素氮(PConclusions.BUN)更高:这些结果表明,以生酮饮食喂养的胱氨酸病动物的肾脏表型明显减轻。
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来源期刊
Journal of The American Society of Nephrology
Journal of The American Society of Nephrology 医学-泌尿学与肾脏学
CiteScore
22.40
自引率
2.90%
发文量
492
审稿时长
3-8 weeks
期刊介绍: The Journal of the American Society of Nephrology (JASN) stands as the preeminent kidney journal globally, offering an exceptional synthesis of cutting-edge basic research, clinical epidemiology, meta-analysis, and relevant editorial content. Representing a comprehensive resource, JASN encompasses clinical research, editorials distilling key findings, perspectives, and timely reviews. Editorials are skillfully crafted to elucidate the essential insights of the parent article, while JASN actively encourages the submission of Letters to the Editor discussing recently published articles. The reviews featured in JASN are consistently erudite and comprehensive, providing thorough coverage of respective fields. Since its inception in July 1990, JASN has been a monthly publication. JASN publishes original research reports and editorial content across a spectrum of basic and clinical science relevant to the broad discipline of nephrology. Topics covered include renal cell biology, developmental biology of the kidney, genetics of kidney disease, cell and transport physiology, hemodynamics and vascular regulation, mechanisms of blood pressure regulation, renal immunology, kidney pathology, pathophysiology of kidney diseases, nephrolithiasis, clinical nephrology (including dialysis and transplantation), and hypertension. Furthermore, articles addressing healthcare policy and care delivery issues relevant to nephrology are warmly welcomed.
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