Hermansky-Pudlak syndrome-2 alters mitochondrial homeostasis in the alveolar epithelium of the lung

BACKGROUND: Mitochondrial dysfunction has emerged as an important player in the pathogenesis of idiopathic pulmonary fibrosis (IPF), a common cause of idiopathic interstitial lung disease in adults. Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder that causes a similar type of...

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Udgivet i:Respir Res
Main Authors: Cuevas-Mora, Karina, Roque, Willy, Shaghaghi, Hoora, Gochuico, Bernadette R., Rosas, Ivan O., Summer, Ross, Romero, Freddy
Format: Artigo
Sprog:Inglês
Udgivet: BioMed Central 2021
Fag:
Research
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7871590/
https://ncbi.nlm.nih.gov/pubmed/33557836
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s12931-021-01640-z
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