muscle
N |
• perineal muscles form normally
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• changes in limb muscle pattern are detected
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Allele Symbol Allele Name Allele ID |
Meox2tm1Vpa targeted mutation 1, Vassilis Pachnis MGI:2674242 |
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Summary |
6 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• perineal muscles form normally
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• changes in limb muscle pattern are detected
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• some of the least affected (about 20%) mutants survive to adulthood, although 80% die before weaning
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• 80% of mutants are severely afflicted by the limb musculature defect and die prior to weaning
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• abnormal limb muscle development, not due to impaired migration of myogenic precursors into the limb bud
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• developmental defect of limb musculature characterized by an overall reduction in muscle mass and elimination of specific muscles
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• variable diameter of skeletal muscle fibers in the fore- and hindlimbs
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• centrally placed nuclei in skeletal muscle fibers of both fore- and hindlimbs
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• neonates show an overall reduction in skeletal muscle mass of the limbs
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• mutants exhibit a limb grasping reflex not observed in controls
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• abnormal extended position of the forelimbs and hindlimbs
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• mutants surviving to adulthood exhibit abnormal gait
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• 10% of mutants exhibit clefting of the secondary palate
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• 10% of mutants exhibit clefting of the secondary palate
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• 10% of mutants exhibit clefting of the secondary palate
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• mice are born in Mendelian ratios but died shortly after birth
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• disrupted somite development, patterning, and differentiation
• absence of ventral (ribs and vertebrae) and dorsal (skeletal muscle) somite derivatives
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• newborn pups present with drastically reduced trunk length
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• rudimentary stump lacking skeletal elements
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• most skeletal muscles are absent or reduced in size
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• mice are born in Mendelian ratios but die shortly after birth
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• disrupted somite development, patterning, and differentiation
• absence of ventral (ribs and vertebrae) and dorsal (skeletal muscle) somite derivatives
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• patterning and specification of the posterior and anterior halves of somites are defective
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• newly formed somites are irregular in shape and fail to epithelialize
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• newly formed somites are irregularly sized
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• newborn pups present with drastically reduced trunk length
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• tail vertebrae are completely absent
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• rudimentary stump lacking skeletal elements
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• myotomes of anterior somites are fused ventrally but separated dorsally, a defect in epithelialization
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• epithelial dermomyotome is absent at E10.5
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• most skeletal muscles are absent or reduced in size in the mutant, including prevertebral muscles of the neck, the epaxial muscles of the trunk, the hypaxial muscles of the trunk including those of the abdominal wall and intercostal muscles
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• the epaxial muscles of the trunk are severely depleted
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• the hypaxial muscles of the trunk including those of the abdominal wall and intercostal muscles are severely depleted
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• mutants lack an axial skeleton
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• occipital skull bones are hypoplastic
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• sternum develops abnormally
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• although centers of ossification are seen at the cervical and thoracic levels, normal ribs are not observed
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• vertebral column is absent and largely replaced by two strips of fused cartilage, in a position corresponding to the neural arches
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• ossified, deformed vertebrae are formed at the cervical and thoracic level but more posterior lumbar vertebrae are present only as cartilage condensations at the position expected of the neural arches
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• tail vertebrae are completely absent
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• segmented organization of the ventral sclerotome is lost and instead a uniform unsegmented mesenchyme is observed
• marker analysis indicates perturbed sclerotome differentiation but not specification
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• brown fat overlying the shoulders muscles is absent
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• occipital skull bones are hypoplastic
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• dorsal root ganglia are irregular in shape and size and fused together
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• spinal nerves are irregular in spacing and direction
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• defects affecting the axial skeleton, however ribs and lumbar vertebrae are normal
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• vertebral defects apparent only at posterior levels
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• tail vertebrae are malformed and fused
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• tail vertebrae are malformed and fused
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• tail vertebrae are fused
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• posterior to the pelvic girdle, poorly differentiated cartilaginous elements are seen in place of vertebrae
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• vertebral bodies are split at the lumbar level
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• tail vertebrae are fused
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 12/10/2024 MGI 6.24 |
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