PMID- 17927973 OWN - NLM STAT- MEDLINE DCOM- 20080324 LR - 20161124 IS - 1095-564X (Electronic) IS - 0012-1606 (Linking) VI - 311 IP - 2 DP - 2007 Nov 15 TI - Menin is required in cranial neural crest for palatogenesis and perinatal viability. PG - 524-37 AB - 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. FAU - Engleka, Kurt A AU - Engleka KA AD - Department of Cell and Developmental Biology and the Cardiovascular Institute, University of Pennsylvania, 1154 BRB II, 421 Curie Blvd, Philadelphia, PA 19104, USA. FAU - Wu, Meilin AU - Wu M FAU - Zhang, Maozhen AU - Zhang M FAU - Antonucci, Nicole B AU - Antonucci NB FAU - Epstein, Jonathan A AU - Epstein JA LA - eng GR - R01 HL 61475/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20070907 PL - United States TA - Dev Biol JT - Developmental biology JID - 0372762 RN - 0 (Men1 protein, mouse) RN - 0 (PAX3 Transcription Factor) RN - 0 (Paired Box Transcription Factors) RN - 0 (Proto-Oncogene Proteins) RN - 0 (Wnt1 Protein) RN - 0 (Wnt1 protein, mouse) RN - 138016-91-8 (Pax3 protein, mouse) SB - IM MH - Abnormalities, Multiple/genetics/pathology MH - Animals MH - Animals, Newborn MH - Cells, Cultured MH - Cleft Palate MH - *Fetal Mortality MH - Humans MH - Mice MH - Mice, Transgenic MH - *Morphogenesis MH - Neural Crest/*metabolism MH - PAX3 Transcription Factor MH - Paired Box Transcription Factors/genetics/metabolism MH - *Palate/embryology/growth & development MH - Proto-Oncogene Proteins/genetics/*metabolism MH - Wnt1 Protein/genetics/metabolism EDAT- 2007/10/12 09:00 MHDA- 2008/03/25 09:00 CRDT- 2007/10/12 09:00 PHST- 2007/07/06 00:00 [received] PHST- 2007/08/24 00:00 [revised] PHST- 2007/08/30 00:00 [accepted] PHST- 2007/10/12 09:00 [pubmed] PHST- 2008/03/25 09:00 [medline] PHST- 2007/10/12 09:00 [entrez] AID - S0012-1606(07)01319-X [pii] AID - 10.1016/j.ydbio.2007.08.057 [doi] PST - ppublish SO - Dev Biol. 2007 Nov 15;311(2):524-37. doi: 10.1016/j.ydbio.2007.08.057. Epub 2007 Sep 7.