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.