Description of human ALS cases used for staining and expression analysisa
| ALS | sALS or fALS | Case description | Age (yr)/sex | Approximate symptom duration (mo) | Autopsy interval (h) | hR23A cytoplasm pattern | hR23A nucleus pattern | hR23B cytoplasm pattern | hR23B nucleus pattern |
|---|---|---|---|---|---|---|---|---|---|
| Case | |||||||||
| JH83 | fALS | SOD1 N193K Onset: left leg | 50/M | 74 | 14.5 | Strong deposits | Strong staining | Strong deposits | Punctate staining |
| JH43 | fALS | SOD1 A4V Onset: LE/left leg | 55/M | 21 | Unknown | Reticular | Punctate staining | Weak deposits | Strong staining |
| JH74 (ALS Case 2 in Fig. 12) | fALS | SOD1 A4V Bulbar onset | 47/M | 15 | 18.5 | Strong and weak deposits | Strong staining | Strong and weak deposits | Punctate staining |
| JH82 | sALS | Onset: LE/left leg | 66/M | 43 | 13 | Absent | Strong staining | Weak deposits | Punctate staining |
| JH73 | sALS | Onset: LE/left foot | 70/F | 36 | 8 | Strong deposits | Strong staining | Punctate and strong deposits | Strong staining |
| JH86 | FTD + fALS (C9ORF72+) | Onset: legs/cognition | 74/M | ∼84 | 32 | Strong deposits | Strong staining | Strong deposits | Weak staining |
| JH79 | sALS | Bulbar onset | 70/F | 28 | 12 | Strong deposits | Strong staining | Strong deposits | Strong straining |
| JH22 | fALS (C9ORF72+) | Onset: UE/left arm | 66/M | 22 | 6 | Punctate throughout | Punctate staining | Absent | Strong staining |
| JH49 | sALS | Onset: UE/right hand | 68/F | 70 | 4 | Strong and weak deposits | Weak staining | Weak deposits | Punctate staining |
| JH88 | FTD + fALS (C9ORF72+) | Bulbar onset | 59/M | 20 | 10 | Reticular | Weak staining | Strong and weak deposits | Weak staining |
| JH07 (ALS Case 1 in Fig. 12) | sALS (C9ORF72+) | Bulbar onset | 67/M | 14 | 8 | Strong deposits | Weak staining | Strong deposits | Strong staining |
| JH80 | sALS | Onset: UE (right) | 56/F | 50 | 21 | Strong deposits | Punctate staining | Strong and weak deposits | Punctate staining |
↵aThe 12 ALS cases from which postmortem spinal cord tissue was taken following autopsy. UE, Upper extremities; LE, lower extremities; sALS, sporadic ALS; fALS, familial ALS (i.e., positive family history). ALS cases in Figure 12C were loaded in Western blot in this order: JH79, JH82, JH74, JH74, JH83.