By unknown mechanisms, the symptoms of @entity746 ( @entity746 ) seem to spread along neuroanatomical pathways to engulf the motor nervous system. The rate at which symptoms spread is one of the primary drivers of disease progression. One mechanism by which @entity746 symptoms could spread is by a prion-like propagation of a toxic misfolded protein from cell to cell along neuroanatomic pathways. Proteins that can transmit toxic conformations between cells often can also experimentally transmit disease between individual organisms. To survey the ease with which @entity4681 ( @entity4681 ) can be transmitted, we injected spinal cord homogenates prepared from paralyzed @entity19 expressing mutant @entity224 dismutase 1 ( @entity182 - @entity24869 and @entity22996 ) into the spinal cords of genetically vulnerable @entity182 @entity19 . From the various models we tested, one emerged as showing high vulnerability. Tissue homogenates from paralyzed @entity24869 @entity19 induced @entity4681 in 6 of 10 @entity19 expressing low levels of @entity22997 - @entity182 fused to yellow fluorescent protein ( @entity22997 -YFP @entity19 ) by 3-11  months, and produced widespread spinal inclusion pathology. Importantly, second passage of homogenates from @entity24869   >   @entity22997 -YFP @entity19 back into newborn @entity22997 -YFP @entity19 induced disease in 4 of 4 @entity19 by 3  months of age. Homogenates from paralyzed @entity19 expressing the @entity22996 variant were among those that transmitted poorly regardless of the strain of recipient transgenic animal injected, a finding suggestive of strain-like properties that manifest as differing abilities to transmit @entity4681 . Together, our data provide a working model for @entity4681 transmission to study the pathogenesis of @entity746 .

Experimental transmissibility of mutant XXXX @entity4681 .

@entity24869 :: ('c|SUB|G|93|A', 'DNAMutation') :: ['G93A'] @entity22997 :: ('p|SUB|G|85|R', 'ProteinMutation') :: ['G85R'] @entity19 :: ('10090', 'Species') :: ['mice', 'transgenic mice'] @entity4681 :: ('MESH:D016472', 'Disease') :: ['MND', 'motor neuron disease'] @entity182 :: ('20655(Tax:10090)', 'Gene') :: ['SOD1'] @entity746 :: ('MESH:D000690', 'Disease') :: ['ALS', 'amyotrophic lateral sclerosis'] @entity224 :: ('MESH:D013481', 'Chemical') :: ['superoxide'] @entity22996 :: ('p|SUB|G|37|R', 'ProteinMutation') :: ['G37R']
@entity182 :: (20655(Tax:10090),Gene) :: ['SOD1']
