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01) and 44% ( .01), respectively, at 12months of age compared to wild-type (WT) mice. The knockin of the hexanucleotide repeat expansion (HRE) caused a 47% loss of motor neurons in the spinal cord ( .001) and 25% (5/2 of female KI rats developed hind limb paralysis at 13 to 24months. Motor defects in KI rats may result from neurotoxicity caused by HRE and the resulting reduction in C9orf72 protein due to haploinsufficiency. These KI rats could be a useful model for investigating the contributions of loss-of-function to neuroto
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