M?llerian inhibiting substance (MIS), also named anti M?llerian hormone (AMH), is a tissue-specific TGF-beta superfamily growth factor. Its expression is restricted to the Sertoli cells of fetal and postnatal testis, and to the granulosa cells of postnatal ovary. The human MIS gene encodes a 553 amino acid residue (aa) prepropeptide containing a signal a sequence (1-24), a pro region (25-455), and the carboxyl-terminal bioactive protein (446-553). MIS induces Mullerian duct (female reproductive tract) regression during sexual differentiation in the male embryo. Posnatally, MIS has been shown to regulate gonadal functions. Like other TGF-beta superfamily members, MIS signals via a heteromeric receptor complex consisting of a type I and a type II receptor serine/threonine kinase (1-5).
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