Likewise, pathological events due to Z-AAT accumulation effects are not limited to the ER, as increased autophagy, mitochondrial injury, cytochrome c release, as well as caspase 3 activation, have been observed in different models of AATD [205,206], as a cellular response that may result in apoptosis [206]. Here, SERPINA1 is linked to alpha 1-antitrypsin deficiency.