mortality/aging
• expected Mendelian ratios of mice are born but homozygotes fail to suckle and subsequently die within the first postnatal day
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muscle
• at E11.5, more progenitor cells have started to differentiate (indicated by increased Myod expression)
• at E12.5 in the limbs, more cells express markers of myogenic differentiation and fewer progenitor cells remain
• at E14.5 in the limbs, fewer muscle progenitor cells are present and those present have reduced proliferative capacity
• at E18.5 all cells are postmitotic unlike in wild-type
• however, the muscle progenitor cells migration is unaffected
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• at E14.5 there is a reduction in the size of limb muscle groups
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• absence of satellite cells in limb muscle at E18.5
• very few satellite cells are seen in intrinsic tongue muscle at E14.5
however, satellite cells can be detected in the distal forelimb
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• muscle fibers in limb muscle have reduced density and the ratio of Myod+ nuclei/fiber is reduced
• however, myofiber diameter is comparable to wild-type
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behavior/neurological
• homozygotes fail to suckle
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