Generation of two Alpha-I antitrypsin deficiency patient-derived induced pluripotent stem cell lines ISRM-AATD-iPSC-1 (HHUUKDi011-A) and ISRM-AATD-iPSC-2 (HHUUKDi012-A)

Autor: Audrey Ncube, Lisa Bewersdorf, Lucas-Sebastian Spitzhorn, Christiane Loerch, Martina Bohndorf, Nina Graffmann, Lea May, Samira Amzou, Malin Fromme, Wasco Wruck, Pavel Strnad, James Adjaye

Publikováno v: Stem Cell Research, Vol 71, Iss , Pp 103171- (2023)

SIX2-positive urine derived renal progenitor cells were isolated from a male and female alpha1-antitrypsin deficiency (AATD) patients both harboring the homozygous PiZZ genotype. The cells were reprogrammed to generate two integration-free induced pl

Externí odkaz: https://doaj.org/article/7319737f42a044e384eca667bc6931da

Phyletic Distribution and Diversification of the Phage Shock Protein Stress Response System in Bacteria and Archaea

Autor: Philipp F. Popp, Vadim M. Gumerov, Ekaterina P. Andrianova, Lisa Bewersdorf, Thorsten Mascher, Igor B. Zhulin, Diana Wolf

Publikováno v: mSystems, Vol 7, Iss 3 (2022)

ABSTRACT Maintaining cell envelope integrity is of vital importance for all microorganisms. Not surprisingly, evolution has shaped conserved protein protection networks that connect stress perception, transmembrane signal transduction, and mediation

Externí odkaz: https://doaj.org/article/52c9d7da50864d559326640017dc0a94

Liver Phenotypes of European Adults Heterozygous or Homozygous for Pi∗Z Variant of AAT (Pi∗MZ vs Pi∗ZZ genotype) and Noncarriers

Autor: Mattias Mandorfer, Nadine T. Gaisa, Christian Trautwein, Jef Verbeek, Helmut Denk, Daniel Neureiter, Elmar Aigner, Julia Kümpers, Heinz Zoller, Barbara Burbaum, LS Moeller, Heike Bantel, Olivier Govaere, Federica Benini, Joanna Chorostowska-Wynimko, Aleksander Krag, Frank Lammert, Jacob George, Katharina Wöran, Thomas Reiberger, Georg Lurje, Marla Gutberlet, Felix Stickel, Lisa Bewersdorf, Michael Trauner, Mohammed Eslam, V Woditsch, Sabina Janciauskiene, Tania Roskams, V Pereira, Johannes Haybaeck, J. Voss, Karim Hamesch, C Lindhauer, Annika Gross, Andreas Geier, Mònica Pons, Malin Fromme, Alexander Teumer, Quentin M. Anstee, Joan Genescà, Matthias C. Reichert, Biaohuan Zhou, Pawel Kuca, Marcin Krawczyk, Carolin V. Schneider, Pavel Strnad, Timm Dirrichs, Christian Datz, Joana Carvão, Robert Bals, Frederik Nevens, Benedikt Schäfer

Publikováno v: Repositório Científico de Acesso Aberto de Portugal
Repositório Científico de Acesso Aberto de Portugal (RCAAP)
instacron:RCAAP
Schneider, C V, Hamesch, K, Gross, A, Mandorfer, M, Moeller, L S, Pereira, V, Pons, M, Kuca, P, Reichert, M C, Benini, F, Burbaum, B, Voss, J, Gutberlet, M, Woditsch, V, Lindhauer, C, Fromme, M, Kümpers, J, Bewersdorf, L, Schäfer, B, Eslam, M, Bals, R, Janciauskiene, S, Carvão, J, Neureiter, D, Zhou, B, Wöran, K, Bantel, H, Geier, A, Dirrichs, T, Stickel, F, Teumer, A, Verbeek, J, Nevens, F, Govaere, O, Krawczyk, M, Roskams, T, Haybaeck, J, Lurje, G, Chorostowska-Wynimko, J, Genesca, J, Reiberger, T, Zoller, H, Lammert, F, Krag, A, George, J, Anstee, Q M, Trauner, M, Datz, C, Gaisa, N T, Denk, H & European Alpha-1 Liver Study Group 2020, ' Liver Phenotypes of European Adults Heterozygous or Homozygous for Pi∗Z Variant of AAT (Pi∗MZ vs Pi∗ZZ genotype) and Noncarriers ', Gastroenterology, vol. 159, no. 2, pp. 534-548.e11 . https://doi.org/10.1053/j.gastro.2020.04.058

BACKGROUND & AIMS: Homozygosity for the Pi∗Z variant of the gene that encodes the alpha-1 antitrypsin peptide (AAT), called the Pi∗ZZ genotype, causes a liver and lung disease called alpha-1 antitrypsin deficiency. Heterozygosity (the Pi∗MZ gen

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d59dc7827cb0387e946e44ea0d463025

Dual proteotoxic stress accelerates liver injury via activation of p62‐Nrf2

Autor: Mayada Metwally, Kanishka Hittatiya, Konrad Kilic, Christian Preisinger, Thomas Berg, Heinz Zoller, Nicole Golob-Schwarzl, Lisa Bewersdorf, Yizhao Luo, Mohammed Eslam, Johannes Haybaeck, Kathrin Wenzel, Alessandra Mangia, Elmar Aigner, Angela Sutton, G.K. Ensari, Rudolf E. Leube, Salvatore Petta, Andre Boonstra, Alessandro Loglio, Jacob George, Pavel Strnad, Maria Lorena Abate, Pietro Lampertico, Deniz Kuscuoglu, Willem P. Brouwer, Pierre Nahon, Annika Gross, Christian Trautwein, Benedikt Schaefer

Publikováno v: Journal of Pathology, 254(1), 80-91. John Wiley & Sons Ltd.

Protein accumulation is the hallmark of various neuronal, muscular, and other human disorders. It is also often seen in the liver as a major protein-secretory organ. For example, aggregation of mutated alpha1-antitrypsin (AAT), referred to as PiZ, is

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b27fcf502fd9f3ecf020c874dddd4c9b
https://doi.org/10.1002/path.5643