title: The Notch-regulated ankyrin repeat protein is required for proper anterior-posterior somite patterning in mice.
abstract: The Notch-regulated ankyrin repeat protein (Nrarp) is a component of a negative feedback system that attenuates Notch pathway-mediated signaling. In vertebrates, the timing and spacing of formation of the mesodermal somites are controlled by a molecular oscillator termed the segmentation clock. Somites are also patterned along the rostral-caudal axis of the embryo. Here, we demonstrate that Nrarp-deficient embryos and mice exhibit genetic background-dependent defects of the axial skeleton. While progression of the segmentation clock occurred in Nrarp-deficient embryos, they exhibited altered rostrocaudal patterning of the somites. In Nrarp mutant embryos, the posterior somite compartment was expanded. These studies confirm an anticipated, but previously undocumented role for the Nrarp gene in vertebrate somite patterning and provide an example of the strong influence that genetic background plays on the phenotypes exhibited by mutant mice.
authors: Center for Molecular Medicine, Maine Medical Center Research Institute, Scarborough, Maine 04074, USA.
authors: Luke T
authors: LT
authors: Krebs
authors: Cara K
authors: CK
authors: Bradley
authors: Christine R
authors: CR
authors: Norton
authors: Jingxia
authors: J
authors: Xu
authors: Kathleen F
authors: KF
authors: Oram
authors: Christa
authors: C
authors: Starling
authors: Michael L
authors: ML
authors: Deftos
authors: Michael J
authors: MJ
authors: Bevan
authors: Thomas
authors: T
authors: Gridley
mesh_terms: D000818:Animals; D017089:Ankyrin Repeat / Q000235:genetics*; D019521:Body Patterning; D005260:Female; D018507:Gene Expression Regulation, Developmental; D018390:Gene Targeting; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008648:Mesoderm; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation; D010641:Phenotype; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D051880:Receptors, Notch / Q000235:genetics / Q000378:metabolism; D015398:Signal Transduction; D019170:Somites / Q000378:metabolism*
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; C499040:Nrarp protein, mouse; D011506:Proteins; D051880:Receptors, Notch