Abstract: Disclosed are a 1-deoxynojirimycin derivative and use thereof. According to the present invention, a variety of 1-deoxynojirimycin derivatives are prepared and obtained through screening. Compared with 1-deoxynojirimycin as a lead compound, the 1-deoxynojirimycin derivative can better bind to an amino acid site that is related to a target protein OPA1, stabilize a binding pocket at a dimer interface, promote the formation of an OPA1 dimer and repair a mitochondrial ultrastructure, thereby significantly saving mitochondrial functions and effectively improving a physiological state of cells. The 1-deoxynojirimycin derivative of the present invention can be used for preparing a drug for treating a disease that is related to unbalanced formation of the OPA1 dimer, and for example, can be used as a potential therapeutic drug for mitochondrial cardiomyopathy and other mitochondrial diseases.