The transmembrane-bound Nrf1 transcription factor, a key member of the CNC-bZIP (cap’n’collar -basic leucine zipper) family, plays a vital role in maintaining intracellular redox balance, proteostasis, endoplasmic reticulum and mitochondrial stability, and so on. Deficiency of Nrf1 in different tissues and organs resulted in a series of diseases in mouse model, including nonalcoholic steatohepatitis, neurodegenerative diseases, diabetes and others. In recent years, new functions of Nrf1 have been gradually revealed in various animal models and clinical findings, especially involving in the process of brown fat tissue thermogenic adaptation (cold adaptation), cholesterol metabolism, glucose metabolism, endoplasmic reticulum stress, and congenital disorder of deglycosylation. Therefore, to gain a better understanding of Nrf1, a briefly review focused on its biological functions was demonstrated in this work.