Kidney-Specific CAP1/Prss8-Deficient Mice Maintain ENaC-Mediated Sodium Balance through an Aldosterone Independent Pathway

Autor: Elodie Ehret, Yannick Jäger, Chloé Sergi, Anne-Marie Mérillat, Thibaud Peyrollaz, Deepika Anand, Qing Wang, Fréderique Ino, Marc Maillard, Stephan Kellenberger, Ivan Gautschi, Roman Szabo, Thomas H. Bugge, Lotte K. Vogel, Edith Hummler, Simona Frateschi

Publikováno v: International Journal of Molecular Sciences, Vol 23, Iss 12, p 6745 (2022)

The serine protease prostasin (CAP1/Prss8, channel-activating protease-1) is a confirmed in vitro and in vivo activator of the epithelial sodium channel ENaC. To test whether proteolytic activity or CAP1/Prss8 abundance itself are required for ENaC a

Externí odkaz: https://doaj.org/article/1cc4224e73f845d493dd991c918734ff

Deletion of the serine protease CAP2/Tmprss4 leads to dysregulated renal water handling upon dietary potassium depletion

Autor: David Hoogewijs, Damien De Bellis, Gilles Crambert, Camille Ansermet, Darko Maric, Petra Klusonova, Denise V. Kratschmar, Robert A. Fenton, Jérémie Canonica, Alex Odermatt, Anna Keppner, Edith Hummler, Chloé Sergi

Publikováno v: Scientific Reports
Keppner, A, Maric, D, Sergi, C, Ansermet, C, De Bellis, D, Kratschmar, D V, Canonica, J, Klusonova, P, Fenton, R A, Odermatt, A, Crambert, G, Hoogewijs, D & Hummler, E 2019, ' Deletion of the serine protease CAP2/Tmprss4 leads to dysregulated renal water handling upon dietary potassium depletion ', Scientific Reports, vol. 9, no. 1, 19540 . https://doi.org/10.1038/s41598-019-55995-x
Scientific Reports, Vol 9, Iss 1, Pp 1-15 (2019)

The kidney needs to adapt daily to variable dietary K+ contents via various mechanisms including diuretic, acid-base and hormonal changes that are still not fully understood. In this study, we demonstrate that following a K+-deficient diet in wildtyp

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c16fb4eed9ec3b0372105f652ef2d621
http://europepmc.org/articles/PMC6925205

Severe hyperkalemia is rescued by low-potassium diet in renal βENaC-deficient mice

Autor: Emilie Boscardin, Johannes Loffing, Dominique Loffing-Cueni, Edith Hummler, Bernard C. Rossier, Chloé Sergi, Romain Perrier, Marc Maillard, Robert Koesters

Publikováno v: Pflugers Archiv, vol. 469, no. 10, pp. 1387-1399

In adulthood, an induced nephron-specific deficiency of αENaC (Scnn1a) resulted in pseudohypoaldosteronism type 1 (PHA-1) with sodium loss, hyperkalemia, and metabolic acidosis that is rescued through high-sodium/low-potassium (HNa + /LK + ) diet. I

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f7a1ef2196951d1c6364d79f30b935ba
https://www.zora.uzh.ch/id/eprint/138075/

Mutations of the Serine Protease CAP1/Prss8 Lead to Reduced Embryonic Viability, Skin Defects, and Decreased ENaC Activity

Autor: Edith Hummler, Nicole Fowler-Jaeger, Chloé Sergi, Carole Planès, Justyna Iwaszkiewicz, Anna Keppner, Nadia Randrianarison, Sumedha Malsure, Anne-Marie Mérillat, Simona Frateschi

Publikováno v: American Journal of Pathology, vol. 181, no. 2, pp. 605-615
The American journal of pathology
AMERICAN JOURNAL OF PATHOLOGY

CAP1/Prss8 is a membrane-bound serine protease involved in the regulation of several different effectors, such as the epithelial sodium channel ENaC, the protease-activated receptor PAR2, the tight junction proteins, and the profilaggrin polypeptide.

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3ac3987ab693ab3bb118ea3000b07385
https://doi.org/10.1016/j.ajpath.2012.05.007

Severe Salt-Losing Syndrome and Hyperkalemia Induced by Adult Nephron-Specific Knockout of the Epithelial Sodium Channel α-Subunit

Autor: Sumedha Malsure, Robert Koesters, Bernard C. Rossier, Dominique Loffing-Cueni, Romain Perrier, Marc Maillard, Mads V. Sorensen, Emilie Boscardin, Simona Frateschi, Johannes Loffing, Edith Hummler, Chloé Sergi

Publikováno v: Perrier, R, Boscardin, E, Malsure, S, Sergi, C, Maillard, M P, Loffing, J, Loffing, D C, Sørensen, M V, Koesters, R, Rossier, B C, Frateschi, S & Hummler, E 2016, ' Severe Salt-Losing Syndrome and Hyperkalemia Induced by Adult Nephron-Specific Knockout of the Epithelial Sodium Channel α-Subunit ', Journal of the American Society of Nephrology, vol. 27, no. 8, pp. 2309-2318 . https://doi.org/10.1681/ASN.2015020154
Journal of the American Society of Nephrology, vol. 27, no. 8, pp. 2309-2318

Systemic pseudohypoaldosteronism type 1 (PHA-1) is a severe salt-losing syndrome caused by loss-of-function mutations of the amiloride-sensitive epithelial sodium channel (ENaC) and characterized by neonatal life-threatening hypovolemia and hyperkale

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bbd0fca94575752321c3e8161d911141
https://pubmed.ncbi.nlm.nih.gov/26701978