Abstract Mitochondria are the foundation of cellular energy metabolism and are crucial for cell growth and development. Mitochondrial dysfunction can disrupt cellular energy metabolism and normal cellular functions, contributing to the onset of related diseases. The functionality of mitochondria is influenced by various associated proteins and molecules, including mitofusin 2, optic atrophy 1, dynamin related protein 1, translocase of the inner membrane 23, translocase of the outer membrane 40, PTEN‐induced kinase 1, reactive oxygen species modulator 1, nicotinamide adenine dinucleotide dehydrogenase, mitochondrial voltage‐dependent anion channel and mitochondrial DNA. We also discussed the role of mitochondrial targeting sequences in mitochondrial proteins. The abnormal expression of these proteins and molecules can impair mitochondrial network remodeling, which is essential for maintaining the quantity and quality of mitochondria and facilitating the exchange of substances between them. This review elucidates the relationship between mitochondrial network remodeling, dysfunction‐induced diseases, and associated proteins and outlines current methods for detecting mitochondrial networks and functions, thereby providing strategies for the study of mitochondrial dysfunction ‐related diseases.