FDX1L (ferredoxin 1-like) is a gene encoding mitochondrial protein whose primary function is to participate in the biosynthesis of iron-sulfur clusters (Fe-S clusters). FDX1L is closely related to the assembly of iron-sulfur clusters and is the second component in the biosynthesis mechanism of iron-sulfur clusters. Studies have shown that FDX1L is widely expressed in human cells, especially in the central nervous system. In addition, mutations in FDX1L can lead to mitochondrial myopathy, manifested by a significant reduction in the activity of respiratory chain complexes I, II, and III. The function of FDX1L is different from that of FDX1 (ferredoxin 1). FDX1 is mainly involved in the synthesis of steroid hormones, while FDX1L focuses on the biosynthesis of iron-sulfur clusters. This functional difference allows FDX1L to play an important role in maintaining homeostasis of iron-sulfur clusters in the cell.