[HTML][HTML] Impaired lysosomal integral membrane protein 2-dependent peroxiredoxin 6 delivery to lamellar bodies accounts for altered alveolar phospholipid content in …
S Kook, P Wang, LR Young, M Schwake… - Journal of Biological …, 2016 - ASBMB
The Hermansky Pudlak syndromes (HPS) constitute a family of disorders characterized by
oculocutaneous albinism and bleeding diathesis, often associated with lethal lung fibrosis.
HPS results from mutations in genes of membrane trafficking complexes that facilitate
delivery of cargo to lysosome-related organelles. Among the affected lysosome-related
organelles are lamellar bodies (LB) within alveolar type 2 cells (AT2) in which surfactant
components are assembled, modified, and stored. AT2 from HPS patients and mouse …
oculocutaneous albinism and bleeding diathesis, often associated with lethal lung fibrosis.
HPS results from mutations in genes of membrane trafficking complexes that facilitate
delivery of cargo to lysosome-related organelles. Among the affected lysosome-related
organelles are lamellar bodies (LB) within alveolar type 2 cells (AT2) in which surfactant
components are assembled, modified, and stored. AT2 from HPS patients and mouse …