PGC-1α is a key transcriptional coactivator regulating energy metabolism, belonging to a family along with PGC-1β and PRC. It is mainly expressed in energetically active tissues such as the liver, heart, and muscles, localized in the cell nucleus, activating multiple signaling pathways including mitochondrial biogenesis, fatty acid oxidation, and glucose metabolism. Through interactions with transcription factors such as PPARs, NRF1, and ERRα, it regulates gene expression. Changes in PGC-1α gene expression are closely associated with various diseases such as obesity, diabetes, cardiovascular diseases, and neurodegenerative diseases. Mutations or alterations in its expression levels can affect energy metabolism, thereby influencing disease development.