Protein odd-skipped-related 1 (OSR1)

OSR1 (Protein odd-skipped-related 1) is a zinc-finger transcription factor and serine/threonine kinase encoded by the OSR1 gene in humans. It plays critical roles in embryonic development of the kidney, heart, palate, and limbs, acting both through regulation of gene expression (as a transcription factor) and by phosphorylation of target proteins in key signaling pathways (as a protein kinase of the Ste20 family)[1][2][3][4][5]. OSR1 is an upstream regulator of cell differentiation and interacts with other transcription factors (e.g., Pax2, Wt1, Lhx1) essential for urogenital formation[1]. In cell signaling, OSR1 controls activity of sodium-potassium-chloride transporters by phosphorylation, a mechanism linked to rare inherited forms of hypertension and other developmental anomalies[2][5]. Loss or dysregulation of OSR1 can lead to kidney agenesis, heart malformations, and abnormal cell fate specification, making it important in disease modeling and a potential therapeutic target for organogenesis-related defects and hypertension[1][4][5].

Other names

Odd-skipped related transcription factor 1ODDOdd-skipped related 1OSR1 kinaseOxidative stress-responsive kinase 1

Experimental inhibitors can disrupt kinase activity, especially affecting the WNK-SPAK/OSR1 signaling axis and downstream ion cotransporters[5].