Exogenous gene transfer of Rab(3)8 small GTPase ameliorates aberrant lung surfactant homeostasis in Ruby rats

BACKGROUND: Rab(3)8 small GTPase regulates intracellular transport in melanocytes and alveolar type II epithelial cells. Ruby rats carrying Rab(3)8 and other gene mutations exhibit oculocutaneous albinism, bleeding diathesis, and hence, are a rat model of human Hermansky-Pudlak syndrome (HPS). We pr...

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Dades bibliogràfiques
Publicat a:Respir Res
Autors principals: Osanai, Kazuhiro, Nakase, Keisuke, Sakuma, Takashi, Nishiki, Kazuaki, Nojiri, Masafumi, Kato, Ryo, Saito, Masatoshi, Fujimoto, Yuki, Mizuno, Shiro, Toga, Hirohisa
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2017
Matèries:
Research
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC5402648/
https://ncbi.nlm.nih.gov/pubmed/28438206
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s12931-017-0549-2
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