Analysis Tools
mortality/aging
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• neonates survive only 3-20 hours after birth
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behavior/neurological
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• no milk was detected in the stomachs of neonates; however, no defect in the suckling reflex was detected
• attempts to bottle feed mice failed apparently due to milk regurgitation and/or infirm breathing
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muscle
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• while cell density, diameter and shape of the mutant fibers appears normal, the sarcoplasmic reticulum of neonates 15-20 hours after birth appeared abnormal with irregular orientation of networks and swollen and/or vacuolated regions
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• the number of triad junctions was reduced in mutant neonate skeletal muscle, although both diad and triad junctions appeared normal in morphology
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• in response to a tail or limb pinch, mutant mice showed normal struggling responses, but had weak voices and did not open mouths widely, suggesting impairment of contractile activity of jaw muscles
• defects in contractility of pharyngoesophageal and diaphragm muscles are suggested to be responsible for the inability of mutant mice to feed
• electrical stimuli at low frequencies developed less contractile forces in mutant muscle than in controls
• extracellular calcium enhanced twitch tension to a greater extent in mutant muscle than in control muscle; neonatal mutant muscle cultured in calcium free conditions exhibited a faster decrease of twitch tension than controls
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