Articles with "nephronophthisis" as a keyword



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Agonists of prostaglandin E2 receptors as potential first in class treatment for nephronophthisis and related ciliopathies

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Published in 2022 at "Proceedings of the National Academy of Sciences of the United States of America"

DOI: 10.1073/pnas.2115960119

Abstract: Significance Juvenile nephronophthisis (NPH) is a renal ciliopathy due to a dysfunction of primary cilia for which no curative treatment is available. This paper describes the identification of agonists of prostaglandin E2 receptors as a… read more here.

Keywords: receptors potential; prostaglandin receptors; potential first; agonists prostaglandin ... See more keywords
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Reducing GEF-H1 Expression Inhibits Renal Cyst Formation, Inflammation, and Fibrosis via RhoA Signaling in Nephronophthisis

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Published in 2023 at "International Journal of Molecular Sciences"

DOI: 10.3390/ijms24043504

Abstract: Nephronophthisis (NPHP) is the most prevalent monogenic disease leading to end-stage renal failure in childhood. RhoA activation is involved in NPHP pathogenesis. This study explored the role of the RhoA activator guanine nucleotide exchange factor… read more here.

Keywords: gef; inflammation fibrosis; expression; rhoa ... See more keywords