A) Healthy alveolar epithelial cells type II (AECII) and b) Hermansky–Pudlak syndrome-associated interstitial pneumonia (HPSIP) AECII. Disturbed intracellular.

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a) Healthy alveolar epithelial cells type II (AECII) and b) Hermansky–Pudlak syndrome-associated interstitial pneumonia (HPSIP) AECII. Disturbed intracellular surfactant transport in AECII occurs. a) Healthy alveolar epithelial cells type II (AECII) and b) Hermansky–Pudlak syndrome-associated interstitial pneumonia (HPSIP) AECII. Disturbed intracellular surfactant transport in AECII occurs. The mature forms of surfactant proteins are blocked inside the late lysosomal compartment of the cell, but not primarily in the endoplasmic reticulum (ER) or Trans-golgi. The AECIIs become swollen due to the accumulation of the mature surfactant proteins, which in turn causes lysosomal stress reactions through activation of cathepsin D, glycerol ceramides and eventually C/EBP homologous protein (CHOP). GlCcer: glucosylceramides; LB: lamellar bodies; MVB: multivesicular bodies; PL: phospholipids; SP: surfactant protein; TM: tubular myelin; ULV: unilamellar vesicles. Andreas Günther et al. Eur Respir Rev 2012;21:152-160 ©2012 by European Respiratory Society