Airway smooth muscle dysfunction in Pompe (Gaa(−/−)) mice

Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase (GAA), an enzyme responsible for hydrolyzing lysosomal glycogen. Deficiency of GAA leads to systemic glycogen accumulation in the lysosomes of skeletal muscle, motor neurons, and smooth muscle. Skeletal mus...

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Detalhes bibliográficos
Publicado no:Am J Physiol Lung Cell Mol Physiol
Main Authors: Keeler, Allison M., Liu, Donghai, Zieger, Marina, Xiong, Lang, Salemi, Jeffrey, Bellvé, Karl, Byrne, Barry J., Fuller, David D., ZhuGe, Ronghua, ElMallah, Mai K.
Formato: Artigo
Idioma:Inglês
Publicado em: American Physiological Society 2017
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5495946/
https://ncbi.nlm.nih.gov/pubmed/28336814
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajplung.00568.2016
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