Abstract
The recessive mutant pudgy in the mouse has a greatly shortened vertebral column with highly irregular fusions between vertebrae and fragments of vertebrae. Ribs and sternum are also involved, but the rest of the skeleton is quite normal. The deformities arise from a defective segmentation. Although the paraxial mesoderm forms somite tissue with an epithelially arranged outer layer, this material either shows only an abortive segmentation into somites or, in the tail, none at all. A belated segmentation into dermomyotomes ultimately takes place, but the sclerotomic material remains continuous and gives rise to erratically abnormal blastemata which then chondrify and ultimately ossify.
Subject
Genetics,General Medicine
Cited by
50 articles.
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1. Introduction;Disordered Vertebral and Rib Morphology in Pudgy Mice;2016
2. Discussion;Disordered Vertebral and Rib Morphology in Pudgy Mice;2016
3. Characterization of the Skeletal Fusion with Sterility (sks) Mouse Showing Axial Skeleton Abnormalities Caused by Defects of Embryonic Skeletal Development;Experimental Animals;2014
4. Notch and disease: A growing field;Seminars in Cell & Developmental Biology;2012-06
5. Mutations in the Notch Pathway Alter the Patterning of Multifidus;The Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology;2011-11-18