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◆ Lab animal2026-09-11

Activating a patient-relevant pathogenic variant of a genetically defined heart disease in a humanized pig model.

Petra Runa-Vochozkova, Michaela Vaskovicova, Josep M Cambra, Andrea Bähr, Eva-Maria Männer, Mayuko Kurome, Valeri Zakhartchenko, Tuna Güngör, Barbara Kessler, Heinke C Heymer, Mara L Corsten, Tarik Bozoglu, Vijayanand Rajendran, Solomon K Yator, Tatjana Dorn, Jessica K Hernandez, Ina M Luksch, Magdalena Harakalova, Peter van Tintelen, Stefan Juhas, Jana Juhasova, Carolina G Cabrera-Gómez, Susanne Zöls, Steffanie Senf, Francesca Stillitano, Joost P G Sluijter, Mathias Ritzmann, Zdenka Ellederova, Motlik Jan, Pieter A Doevendans, David Drutovic, Pieter C Glijnis, Eckhard Wolf, Christian Kupatt, Nikolai Klymiuk

原始摘要(英文原文)· Original abstract
Complex diseases such as progressive cardiomyopathies are often insufficiently recapitulated in small animals or in vitro models. Large animal species such as pigs provide a valuable alternative, but constitutive genetic manipulation has not yet been applied to pigs in an effective manner, mainly due to biological and logistical limitations. Here we describe the generation of a humanized pig model for phospholamban-mediated cardiomyopathy and compare different methods for activating a pathogenic R14del mutation by Cre-mediated recombination. Both Cre treatment of pig primary cells before somatic cell nuclear transfer as well as microinjection of Cre-encoding mRNA into zygotes were similarly efficient in delivering piglets with an activated R14del mutation. Alternatively, administration of Cre-encoding adeno-associated virus into piglets was sufficient, albeit to a varying extent. Together, we describe a highly effective process to establish complex inducible genetic traits in pig and demonstrate that the lack of Cre-driver lines can be compensated by various interventions during reproduction or postnatally.
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Activating a patient-relevant pathogenic variant of a genetically defined heart disease in a humanized pig model. — 科研速览 Science Skim