Table 1.
Cases utilized for this study
| Age | Gender | PMI (hours) | Percentage of Motor Neurons with Nuclear CRABP-II Localization | Percentage of Motor Neurons with Increased RARβ Nuclear Localization | |
|---|---|---|---|---|---|
| SALS1 | 45 | M | 11 | 40% (4 of 10) | 55% (6 of 11) |
| SALS2 | 51 | M | 7 | 50% (4 of 8) | 60% (6 of 10) |
| SALS3 | 45 | M | 7 | 82% (9 of 11) | 64% (7 of 11) |
| SALS4 | 62 | M | 7 | 70% (7 of 10) | 14% (1 of 7) |
| SALS5 | 73 | M | 6 | 82% (9 of 11) | 55% (6 of 11) |
| SALS6 | 80 | F | 4 | 64% (9 of 14) | 88% (22 of 25) |
| SALS7 | 82 | M | 5 | 64% (9 of 14) | 73% (22 of 30) |
| SALS8 | 72 | F | 9 | 50% (4 of 8) | 70% (7 of 10) |
| SALS9 | 56 | M | 3 | 63% (5 of 8) | 62% (8 of 13) |
| SALS10 | 63 | F | 5 | 67% (6 of 9) | 18% (2 of 11) |
| SALS11 | 59 | M | 4 | 67% (8 of 12) | 33% (5 of 15) |
| SALS12* | 43 | M | 6 | 70% (7 of 10) | 86% (6 of 7) |
| SALS13* | 67 | M | 4 | 41% (7 of 17) | 13% (2 of 16) |
| SALS14 | 51 | M | 4 | 78% (7 of 9) | 58% (7 of 12) |
| SALS15 | 40 | M | 6 | 38% (3 of 8) | 44% (4 of 9) |
| SALS16 | 77 | F | 4 | 89% (8 of 9) | 50% (5 of 10) |
| SALS17 | 69 | M | 5 | 80% (8 of 10) | 56% (5 of 9) |
| SALS18 | 60 | F | 2 | 70% (7 of 10) | 27% (3 of 11) |
| SALS19 | 50 | F | 7 | 88% (7 of 8) | 73% (8 of 11) |
| SALS20 | 60 | F | 4 | 78% (7 of 9) | 50% (5 of 10) |
| FALS1 | 45 | M | 3 | 17% (2 of 12) | 8% (1 of 12) |
| FALS2 | 48 | F | 5 | 17% (2 of 12) | 20% (2 of 10) |
| FALS3 | 55 | M | 14 | 56% (5 of 9) | 20% (2 of 10) |
| FALS4* | 63 | F | 4 | 50% (4 of 8) | 20% (2 of 10) |
| CON1 | 54 | M | 6 | 26% (6 of 23) | 13% (2 of 15) |
| CON2 | 51 | F | 5 | 38% (3 of 8) | 18% (2 of 11) |
| CON3 | 76 | M | 13 | 31% (4 of 13) | 29% (9 of 31) |
| CON4 | 57 | M | 2 | 25% (3 of 12) | 4% (2 of 47) |
| CON5 | 58 | F | 5 | 73% (8 of 11) | 25% (6 of 24) |
| CON6 | 82 | F | 5 | 11% (1 of 9) | 19% (4 of 21) |
| CON7 | 57 | F | 11 | ND | 21% (7 of 34) |
| CON8 | 95 | M | 20 | 50% (5 of 10) | 0% (0 of 7) |
| CON9 | 53 | F | 4 | 20% (2 of 10) | 6% (1 of 16) |
The age and post-mortem interval (PMI) in hours (average SALS 5.50 hours; average FALS 6.5 hours; average control 7.89 hours) are indicated for the SALS (60.25 years), FALS (52.75 years) and control (64.78 years) cases. 17 of 20 SALS cases exhibited increased nuclear localization of RARβ as compared to controls and FALS.
*
signifies presence of the C9ORF72 repeat expansion within the subject.
ND = not determined