Human MIS RII Antibody

Human MIS RII (Mullerian inhibiting substance type II receptor), also known as AMHRII (anti-Mullerian hormone type II receptor), is an 82 kDa serine/threonine receptor with a single transmembrane domain that belongs to the family of type II receptors of the TGF-beta superfamily (1). The MIS RII precursor is 573 amino acids in length, with a 17 amino acid (aa) signal sequence, a 127 aa extracellular region that also contains two potential N-linked glycosylation sites, a 26 aa transmembrane region, and a 403 aa cytoplasmic region that contains the serine/threonine kinase domain (1). Human MIS RII shares 82%, 78%, and 77% aa sequence identity with rabbit, mouse, and rat MIS RII, respectively. It is expressed in the mesenchyme surrounding the fetal Mullerian duct, in fetal and postnatal granulosa cells, and in Sertoli cells (1-6). MIS RII is a receptor for Mullerian inhibitor substance (MIS), also known as anti-Mullerian hormone (AMH), which is responsible for regression of the Mullerian duct, the anlagen of the uterus, Fallopian tubes, and upper vagina in male fetuses (1-6). Mutations in MIS RII result in persistent Mullerian duct syndrome (PMDS), an extremely rare form of pseudohermaphroditism (5, 6).