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Sperm-associated antigen 17 (SPAG17) is a large structural protein (~250 kDa) that is an essential component of the central pair apparatus of axonemal microtubules in cells with 9+2 motile cilia or flagella[2][3][1]. SPAG17 plays a critical role in the proper assembly and function of motile cilia, being crucial for airway mucociliary clearance, normal brain ventricular physiology, and sperm motility[1][2][3][4]. Mutations or knockout of SPAG17 cause loss or impairment of ciliary motility, leading to primary ciliary dyskinesia, male infertility, hydrocephalus, and severe respiratory distress (notably in mice, neonatal lethality occurs within hours due to airway dysfunction)[1][2][3]. SPAG17 is also important for the development and maintenance of skeletal structures, with variants implicated in human height and limb abnormalities[3]. The protein interacts with other central pair proteins such as SPAG6 and SPAG16, and is required for the proper structure of the sperm head and flagellum[2][3][1]. Currently, there are no known small-molecule drugs targeting SPAG17, nor is it a therapeutic target in the sense of druggable receptors or enzymes[3].
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