Difficulty: Medium/Hard-- Mitochondria play a key role in producing energy for the cell and mitochondrial dysfunction is linked with many different diseases such as Leber's hereditary optic neuropathy, Friedreich's ataxia, Wilson's disease, hereditary spastic paraplegia, and more. Deficiency in N-glycanase 1 is expected to result in the aggregation of N-glycosylated proteins. Protein aggregation is an expected trigger of the ER stress response which can affect mitochondrial function. Does the literature surrounding protein misfolding and mitochondrial function hold clues about the mechanism of disease progression in NGLY1 deficiency? Mark the docs to help us find out!