9.B.317. The Complex I Integral Membrane Chaparone, TMEM126 (TMEM126) Family
Cell stress such as hypoxia elicits adaptive responses on cells. An assembly factor for mitochondrial complex I is transmembrane protein 126B (TMEM126B). Fuhrmann et al. 2018 identified changes in complex I abundance under chronic hypoxia, in association with impaired substrate-specific mitochondrial respiration. Complexome profiling of isolated mitochondria of the human leukemia monocytic cell line THP-1 revealed deficits in complex I assembly. They demonstrated a selective decrease of TMEM126B under chronic hypoxiadue to the proteolytic degradation of TMEM126B.