Fig. 7
From: Hermansky-Pudlak syndrome-2 alters mitochondrial homeostasis in the alveolar epithelium of the lung
A cartoon depicting various aspects by which HPS-2 alters mitochondrial homeostasis in the alveolar epithelium. Ap3b1 mutation/deficiency alters multiple homeostatic processes in mitochondria including bioenergetics, TCA cycle, mitochondrial biogenesis, redox balance and proteotoxic stress responses. To date, the mechanisms leading to the wide array of cellular changes of HPS-2 alveolar epithelium remain unknown. UPRmt mitochondrial unfolded protein response, OCR oxygen consumption rate; ETC electron transport chain; TCA tricarboxylic acid cycle; ROS reactive oxygen species, PGC1α peroxisome proliferator-activated receptor gamma coactivator 1-alpha; NRF1 nuclear respiration factor 1; TFAM mitochondrial transcription factor A; ATF5 activating transcription factor 5, AP3B1 adaptor related protein complex 3 subunit beta 1