ZEB2/SIP1: Proteins and Enzymes

SIP1 (Smad-interacting protein 1), also called Zeb-2 (Zinc finger E-box-binding homeobox 2), is a nuclear transcription factor. SIP1 contains seven C2H2-type zinc finger domains, a Smad-MHZ binding domain, an atypical homeobox domain, and phosphorylation, sumoylation and acetylation sites. SIP1 is highly expressed in postmitotic neocortical cells and influences cell fate decisions in embryonic brain development. Point mutations causing underexpression of SIP1 are associated with Mowat-Wilson syndrome (MWIS), also known as Hirschprung disease mental retardation syndrome.