Dataset Information

Finding the Unicorn, a New Mouse Model of Midfacial Clefting.

ABSTRACT: Human midfacial clefting is a rare subset of orofacial clefting and in severe cases, the cleft separates the nostrils splitting the nose into two independent structures. To begin to understand the morphological and genetic causes of midfacial clefting we recovered the Unicorn mouse line. Unicorn embryos develop a complete midfacial cleft through the lip, and snout closely modelling human midfacial clefting. The Unicorn mouse line has ethylnitrosourea (ENU)-induced missense mutations in Raldh2 and Leo1. The mutations segregate with the cleft face phenotype. Importantly, the nasal cartilages and surrounding bones are patterned and develop normal morphology, except for the lateral displacement because of the cleft. We conclude that the midfacial cleft arises from the failure of the medial convergence of the paired medial nasal prominences between E10.5 to E11.5 rather than defective cell proliferation and death. Our work uncovers a novel mouse model and mechanism for the etiology of midfacial clefting.

SUBMITTER: Lantz B

PROVIDER: S-EPMC7016607 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Finding the Unicorn, a New Mouse Model of Midfacial Clefting.

Lantz Brandi B White Casey C Liu Xinyun X Wan Yong Y Gabriel George G Lo Cecilia W Y CWY Szabo-Rogers Heather L HL

Genes 20200111 1

Human midfacial clefting is a rare subset of orofacial clefting and in severe cases, the cleft separates the nostrils splitting the nose into two independent structures. To begin to understand the morphological and genetic causes of midfacial clefting we recovered the Unicorn mouse line. Unicorn embryos develop a complete midfacial cleft through the lip, and snout closely modelling human midfacial clefting. The Unicorn mouse line has ethylnitrosourea (ENU)-induced missense m ...[more]

PMID: 31940751

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Project description:Following facial prominence fusion, anterior-posterior (A-P) elongation of the palate is a critical aspect of palatogenesis and integrated midfacial elongation. Reciprocal epithelial-mesenchymal interactions drive secondary palate elongation and periodic signaling center formation within the rugae growth zone (RGZ). However, the relationship between RGZ dynamics and the morphogenetic behavior of underlying palatal bone mesenchymal precursors has remained enigmatic. Our results indicate that cellular activity at the RGZ simultaneously drives rugae formation and elongation of the maxillary bone primordium within the anterior secondary palate, which more than doubles in length prior to palatal shelf fusion. The first formed palatal ruga, found just posterior to the RGZ, represents a consistent morphological boundary between anterior and posterior secondary palate bone precursors, being found at the future maxillary-palatine suture. These results suggest a model where changes in RGZ-driven A-P growth of the anterior secondary palate may produce interspecies and intraspecies differences in facial prognathism and differences in the proportional contribution of palatal segment-associated bones to total palate length. An ontogenetic comparison of three inbred mouse strains indicated that while RGZ-driven growth of the anterior secondary palate is critical for early midfacial outgrowth, subtle strain-specific bony contributions to adult palate length are not present until after this initial palatal growth period. This multifaceted illustration of normal midfacial growth dynamics confirms a one-to-one relationship between palatal segments and upper jaw bones during the earliest stages of palatal growth, which may serve as the basis for evolutionary change in upper jaw morphology. Additionally, identified mouse strain-specific differences in palatal segment elongation provide a useful foundation for understanding the impact of background genetic effects on facial morphogenesis.

Dataset Information

Finding the Unicorn, a New Mouse Model of Midfacial Clefting.

Publications

Finding the <i>Unicorn</i>, a New Mouse Model of Midfacial Clefting.

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