The current study showed that AGK deficiency facilitated the translocation of mitochondrial respiratory chain complex I subunits, NDUFS2 and NDUFA10, to lysosomes and decreased the protein levels, therefore causing the deficiency of mitochondrial respiratory chain complex I. Therefore, the reduction of NDUFS2 and NDUFA10 in NASH underwent a lysosome degradation dependent pathway. The gene discussed is NDUFV1; the disease is metabolic dysfunction-associated steatohepatitis.