Proteomics

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A scalable, clinically severe pig model for Duchenne muscular dystrophy

ABSTRACT: Large animal models for Duchenne muscular dystrophy (DMD) are indispensible for preclinical evaluation of novel diagnostic procedures and treatment strategies. To evaluate functional consequences of Duchenne muscular dystrophy (DMD) in skeletal muscle and myocardium, we used a new genetically engineered dystrophin KO pig model displaying hallmarks of human DMD. Heart and skeletal muscle tissue samples of DMD pigs and wild-type (WT) controls at three different ages were analyzed by label-free proteomics.

INSTRUMENT(S): Q Exactive HF

ORGANISM(S): Sus Scrofa Domesticus (domestic Pig)

TISSUE(S): Heart, Skeletal Muscle

DISEASE(S): Duchenne Muscular Dystrophy

SUBMITTER: Thomas Fröhlich  

LAB HEAD: Thomas Fröhlich

PROVIDER: PXD027772 | Pride | 2021-12-01

REPOSITORIES: Pride

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A scalable, clinically severe pig model for Duchenne muscular dystrophy.

Stirm Michael M   Fonteyne Lina Marie LM   Shashikadze Bachuki B   Lindner Magdalena M   Chirivi Maila M   Lange Andreas A   Kaufhold Clara C   Mayer Christian C   Medugorac Ivica I   Kessler Barbara B   Kurome Mayuko M   Zakhartchenko Valeri V   Hinrichs Arne A   Kemter Elisabeth E   Krause Sabine S   Wanke Rüdiger R   Arnold Georg J GJ   Wess Gerhard G   Nagashima Hiroshi H   Hrabĕ de Angelis Martin M   Flenkenthaler Florian F   Kobelke Levin Arne LA   Bearzi Claudia C   Rizzi Roberto R   Bähr Andrea A   Reese Sven S   Matiasek Kaspar K   Walter Maggie C MC   Kupatt Christian C   Ziegler Sibylle S   Bartenstein Peter P   Fröhlich Thomas T   Klymiuk Nikolai N   Blutke Andreas A   Wolf Eckhard E  

Disease models & mechanisms 20211216 12

Large-animal models for Duchenne muscular dystrophy (DMD) are crucial for the evaluation of diagnostic procedures and treatment strategies. Pigs cloned from male cells lacking DMD exon 52 (DMDΔ52) exhibit molecular, clinical and pathological hallmarks of DMD, but die before sexual maturity and cannot be propagated by breeding. Therefore, we generated female DMD+/- carriers. A single founder animal had 11 litters with 29 DMDY/-, 34 DMD+/- as well as 36 male and 29 female wild-type offspring. Bree  ...[more]

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