Skeletal, cardiac, and respiratory muscle function and histopathology in the P448Lneo− mouse model of FKRP-deficient muscular dystrophy

Abstract Background Fukutin-related protein (FKRP) mutations are the most common cause of dystroglycanopathies known to cause both limb girdle and congenital muscular dystrophy. The P448Lneo− mouse model has a knock-in mutation in the FKRP gene and develops skeletal, respiratory, and cardiac muscle...

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Autori principali: Qing Yu, Melissa Morales, Ning Li, Alexander G. Fritz, Ren Ruobing, Anthony Blaeser, Ershia Francois, Qi-Long Lu, Kanneboyina Nagaraju, Christopher F. Spurney
Natura: Artigo
Lingua:Inglês
Pubblicazione: BMC 2018-04-01
Serie:Skeletal Muscle
Soggetti:
Limb-girdle muscular dystrophy
Congenital muscular dystrophy
Fukutin related protein (FKRP)
P448Lneo− mice
Echocardiography
Plethysmography
Accesso online:http://link.springer.com/article/10.1186/s13395-018-0158-x
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