Figure 5

Paraspeckle formation is frequently observed in an early phase of ALS. A. A scheme of TDP-43 aggregation patterns in ALS is presented. Observations detected at 647 nm are added to distinguish the TDP-43-positive signals from autofluorescence produced by lipofuscin granules in the cytoplasm (shown as an asterisk). Scale bars, 10 μm. B. Stages of motor neurons in sporadic ALS and control cases are shown according to the definitions in Table 2. The number on the bar graph represents the proportion of motor neurons at each stage in a case. C. NEAT1_2-positive rates in the spinal motor neurons at stage 0 are indicated (ALS vs. control). Each triangle or circle represents an individual ALS (Pt) or control (C) patient, respectively. The actual value of percentage in each case is shown in parenthesis. Bars represent the mean ± s.d.; that is, 85.6 ± 11.0% in ALS cases and 35.1 ± 22.6% in control cases (n = 6 per group). *P<0.001. D. NEAT1_2-positive rates in spinal motor neurons at each stage are plotted. Mean ± s.d. were 54.0 ± 28.6% at stage I, 15.1 ± 6.1% at stage II, and 0.0 ± 0.0% at stage III (n = 6 per group). *P<0.05, **P<0.01, ***P<0.001.