title: Deficient Alk3-mediated BMP signaling causes prenatal omphalocele-like defect.
abstract: BMP signaling plays important roles in many embryonic developmental processes. Alk3 is one of two BMP type I receptors that transduces BMP signal from the cell surface into cell. Conventional knockout of Alk3 resulted in early embryonic lethality around E7.5-E9.5. In this study, we have generated embryonic mesoderm-specific Alk3 conditional knockout by crossing Dermo1-Cre and floxed Alk3 mice. Abrogation of Alk3-mediated BMP signaling in this mouse resulted in severe defect of secondary ventral body wall formation, replicating the omphalocele phenotype in human. Our finding suggests that Alk3 plays an essential role in the formation of embryonic ventral abdominal wall, and abrogation of BMP signaling activity due to gene mutations in its signaling components could be one of the underlying causes of omphalocele at birth.
authors.affiliation: Developmental Biology Program, Department of Surgery, Childrens Hospital Los Angeles, 4650 Sunset Boulevard, MS 35, Los Angeles, CA 90027, USA.
authors.forename: Jianping
authors.initials: J
authors.lastname: Sun
authors.forename: Yi-Hsin
authors.initials: YH
authors.lastname: Liu
authors.forename: Hui
authors.initials: H
authors.lastname: Chen
authors.forename: Manuel P
authors.initials: MP
authors.lastname: Nguyen
authors.forename: Yuji
authors.initials: Y
authors.lastname: Mishina
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Upperman
authors.forename: Henri R
authors.initials: HR
authors.lastname: Ford
authors.forename: Wei
authors.initials: W
authors.lastname: Shi
mesh_terms: D000818:Animals; D019521:Body Patterning; D052005:Bone Morphogenetic Protein Receptors, Type I / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D006554:Hernia, Umbilical / Q000196:embryology* / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D015398:Signal Transduction
chemical_list: C498212:Bmpr1a protein, mouse; D052005:Bone Morphogenetic Protein Receptors, Type I