Fig. 8.

ALS-associated mutation within FUS/TLS 3’UTR inhibits the ability for miR-b2122 to reduce firefly activity. HEK293T cells were co-transfected with either the pmirGLO plasmid containing the wild-type FUS/TLS 3’UTR, or the mutated form, and either with or without miR-b2122. a ALS-associated mutation (*c.108 C > T) affects the +2 binding site of the miR-b2122 MRE. b ALS-associated mutation within the FUS/TLS 3’UTR inhibits miR-b2122 from reducing firefly activity. Firefly expression was normalized to renilla expression, and then further normalized to account for the effect miR-b2122 on the pmirGLO vector itself to determine the miRNAs exact effect on the 3’UTR. c Western blot of FUS/TLS protein expression in the spinal cord of sALS cases versus control subjects. d Quantification of western blot. Data is expressed as sample mean ± SEM, and significance was determined using a Student’s t-test (** = p < 0.01)