AIF1 and amyotrophic lateral sclerosis: While hFUS−/− and hFUS+/− mice appear normal for the entire time frame considered, hFUS+/+ mice show clear signs of motor phenotypes and ALS pathology, including a decreased number of motor neurons in the lumbar spinal cord, increased expression of astroglial and microglial inflammatory markers, such as GFAP and Iba1, inability to gain weight, signs of progressive hind limb paralysis and early lethality (Supplementary Figure 1).