Hemizygous PFN1G118V transgenic mice exhibited many pathological features of ALS, including loss of lower and upper MNs, loss of MNJs, aggregation of the mutant profilin 1 protein, abnormally ubiquitinated proteins, increase in nuclear staining of phosphorylated TDP-43 in the spinal cord, fragmented mitochondria, glial cell activation, muscle atrophy, weight loss, and reduced survival [175]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.