Analysis Tools
mortality/aging
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• 90% of mutants die within 1 month of birth
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behavior/neurological
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• starting a few days after birth mutants develop progressive defects in motor function including an inability to climb on inclined surfaces or cling to a round bar
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muscle
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• starting a few days after birth mutants develop progressive muscle atrophy
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• starting a few days after birth mutants develop progressive muscle weakness
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nervous system
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• at E13.5 and E14.5 the subcutaneous nerves in mutants were thinner with an abnormal arborization pattern
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• at E13.5 few or no Schwann cell precursors are seen in the distal part of the developing subcutaneous or muscular innervations however by E16.5 Schwann cell numbers have returned to normal
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• mutants have decreased intramuscular innervation
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• neuromuscular junctions in the abdominal, soleus, and gastrocnemius muscles and the diaphragm appear immature in mutants at P21
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• at E10.5 transient abnormalities including decreased vagus nerve roots, fusion of nerves IX and X, or absence of nerve IX are seen however by E11.5 cranial nerve patterning has resembles that in controls
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• at P1 the sciatic nerve is abnormally thin and becomes progressively thinner by P21
• mutants nerves contain myelinated motor axons and large clusters of unsorted axons of varying diameters with few myelinated sensory axons
• at P21 some extracellular matrix free regions are seen in mutants
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• only a few myelinated sensory axons are seen at P21 in the sciatic nerve
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cellular
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• at E13.5 few or no Schwann cell precursors are seen in the distal part of the developing subcutaneous or muscular innervations however by E16.5 Schwann cell numbers have returned to normal
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