Systemic ASBT inactivation protects against liver damage in obstructive cholestasis in mice

Autor: Roni F. Kunst, Dirk R. de Waart, Frank Wolters, Suzanne Duijst, Esther W. Vogels, Isabelle Bolt, Joanne Verheij, Ulrich Beuers, Ronald P.J. Oude Elferink, Stan F.J. van de Graaf

Publikováno v: JHEP Reports, Vol 4, Iss 11, Pp 100573- (2022)

Background & Aims: Non-absorbable inhibitors of the apical sodium-dependent bile acid transporter (ASBT; also called ileal bile acid transporter [IBAT]) are recently approved or in clinical development for multiple cholestatic liver disorders and lea

Externí odkaz: https://doaj.org/article/44bd0d993371444b94902a2e94c0bc06

Reduced spontaneous itch in mouse models of cholestasis

Autor: Jacqueline Langedijk, Ruth Bolier, Dagmar Tolenaars, Lysbeth ten Bloemendaal, Suzanne Duijst, Dirk de Waart, Ulrich Beuers, Piter Bosma, Ronald Oude Elferink

Publikováno v: Scientific Reports, Vol 11, Iss 1, Pp 1-13 (2021)

Abstract Pruritus is one of the most distressing symptoms in cholestatic patients. Plasma autotaxin (ATX) activity correlates with the severity of pruritus in cholestatic patients, but the pathophysiology is unclear. To study pruritus in mice, we mea

Externí odkaz: https://doaj.org/article/8a91086451c548c488f3ed16188f6c17

Low efficacy of recombinant SV40 in Ugt1a1-/- mice with severe inherited hyperbilirubinemia.

Autor: Xiaoxia Shi, Giulia Bortolussi, Lysbeth Ten Bloemendaal, Suzanne Duijst, Andrés F Muro, Piter J Bosma

Publikováno v: PLoS ONE, Vol 16, Iss 4, p e0250605 (2021)

In contrast to AAV, Simian Virus 40 (rSV40) not inducing neutralizing antibodies (NAbs) allowing re-treatment seems a promising vector for neonatal treatment of inherited liver disorders. Several studies have reported efficacy of rSV40 in animal mode

Externí odkaz: https://doaj.org/article/a08eb17d84844a7a8088f691c132c137

Efficacy of AAV8-hUGT1A1 with Rapamycin in neonatal, suckling, and juvenile rats to model treatment in pediatric CNs patients

Autor: Piter J. Bosma, Andrés F. Muro, Giulia Bortolussi, Xiaoxia Shi, Sem J. Aronson, Dirk R. de Waart, Ronald P.J. Oude Elferink, Suzanne Duijst, Fanny Collaud, Robert S. Bakker, Lysbeth ten Bloemendaal, Giuseppe Ronzitti, Federico Mingozzi

Publikováno v: Molecular Therapy: Methods & Clinical Development, Vol 20, Iss, Pp 287-297 (2021)
Molecular therapy. Methods & clinical development, 20, 287-297. Nature Publishing Group
Molecular Therapy. Methods & Clinical Development

A clinical trial using adeno-associated virus serotype 8 (AAV8)-human uridine diphosphate glucuronosyltransferase 1A1 (hUGT1A1) to treat inherited severe unconjugated hyperbilirubinemia (Crigler-Najjar syndrome) is ongoing, but preclinical data sugge

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::aee63f1483121a3b8be1553a09080dfd
http://www.scopus.com/inward/record.url?scp=85099261102&partnerID=8YFLogxK

Blocking Sodium‐Taurocholate Cotransporting Polypeptide Stimulates Biliary Cholesterol and Phospholipid Secretion in Mice

Autor: Reinout L.P. Roscam Abbing, Suzanne Duijst, Folkert Kuipers, Coen C. Paulusma, Rick Havinga, Joanne M. Donkers, Ronald P.J. Oude Elferink, Stan F.J. van de Graaf, Albert K. Groen, Davor Slijepcevic, Johan Kuiper

Publikováno v: Hepatology
Hepatology, 71(1), 247-258. Wiley
Hepatology, 71(1), 247-258
Hepatology (Baltimore, Md.)
Hepatology (Baltimore, Md.), 71(1), 247-258. John Wiley and Sons Ltd

Active secretion of bile salts into the canalicular lumen drives bile formation and promotes biliary cholesterol and phospholipid output. Disrupting hepatic bile salt uptake, by inhibition of sodium‐taurocholate cotransporting polypetide (NTCP; Slc

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0c8925e3395df97e321cdbc1d9aae511
https://doi.org/10.1002/hep.30792