TLX (Tailless) is a member of the orphan nuclear receptor family, containing a DNA-binding domain and a ligand-binding domain. It is mainly expressed in the central nervous system, especially in neural stem cells and developing neurons. TLX participates in biological responses such as self-renewal, differentiation, and neurodevelopment of neural stem cells by regulating the expression of downstream genes. TLX interacts with multiple signaling pathways, such as the Wnt and Notch pathways, to regulate cell fate decisions. The specific expression of the TLX gene is associated with its important role in neurodevelopment, and its mutations or abnormal expression are linked to neurodevelopmental disorders and certain neurodegenerative diseases.