SF-1 is a transcriptional activator that essential for sexual differentiation and formation of the primary steroidogenic tissues. It also activated by the phosphorylation of NR5A1 by HIPK3 leading to increased steroidogenic gene expression upon cAMP signaling pathway stimulation. SF-1 is highly expressed in the adrenal cortex, ovary, testis, spleen. SF-1 is generally used for diagnosis of sex cord-stromal tumors and differantial diagnosis of adrenocortical adenoma and pheochromocytoma.