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Dev Biol. 2007 Nov 15;311(2):524-37. Epub 2007 Sep 7.

Menin is required in cranial neural crest for palatogenesis and perinatal viability.

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  • 1Department of Cell and Developmental Biology and the Cardiovascular Institute, University of Pennsylvania, 1154 BRB II, 421 Curie Blvd, Philadelphia, PA 19104, USA.


Menin is a nuclear protein encoded by a tumor suppressor gene that is mutated in humans with multiple endocrine neoplasia type 1 (MEN1). Menin functions as a component of a histone methyltransferase complex that regulates expression of target genes including the cell cycle inhibitor p27(kip1). Here, we show that menin plays a previously unappreciated and critical role in cranial neural crest. Tissue-specific inactivation of menin in Pax3- or Wnt1-expressing neural crest cells leads to perinatal death, cleft palate and other cranial bone defects, which are associated with a decrease in p27(kip1) expression. Deletion of menin in Pax3-expressing somite precursors also produces patterning defects of rib formation. Thus, menin functions in vivo during osteogenesis and is required for palatogenesis, skeletal rib formation and perinatal viability.

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