Table 1.
Summary of studies examining the presence of TDP-43 CTF-25 in postmortem brain and spinal cord tissue from people with ALS and FTLD-TDP.
| Disease | Tissue Type | Presence of CTFs | Major Findings | |
|---|---|---|---|---|
| ALS | Brain | + | • Increased CTFs compared to healthy controls in frontal and temporal cortices (Hasegawa et al., 2008; Tsuji et al., 2012a; Tsuji et al., 2012b; Kwong et al., 2014; Smethurst et al., 2016), hippocampus, substantia nigra, striatum, medulla and pons (Tsuji et al., 2012a), and in studies where brain region was not specified (Inukai et al., 2008; Zhang et al., 2009; Kametani et al., 2016) | |
| - | • No CTFs in temporal cortex or cerebellum (Neumann et al., 2006; Tsuji et al., 2012a) | |||
| • Low levels of CTFs in the frontal cortex of some patients (Neumann et al., 2006) | ||||
| Spinal Cord | ? | • CTFs faintly detected in the lumbar spinal cord, less abundant than full-length TDP-43 (Tsuji et al., 2012a; Smethurst et al., 2016) | ||
| • Low levels of CTFs in both ALS and healthy control tissue (Giordana et al., 2010) | ||||
| • CTFs observed in one patient with a TARDBP mutation (Q343R) but not in sporadic ALS patients (Yokoseki et al., 2008) | ||||
| • 35 kDa fragment was detected in the ALS spinal cord (Xiao et al., 2015) | ||||
| - | • No CTFs (immunoblotting) in lumbar spinal cord (Neumann et al., 2006, 2009; Igaz et al., 2008; Uchida et al., 2012) | |||
| • No CTFs (immunohistochemistry) in spinal cord ventral horn (Igaz et al., 2008; Kwong et al., 2014) | ||||
| FTLD-TDP | Type A | Brain | + | • CTFs detected in cortex (Neumann et al., 2006; Igaz et al., 2008; Arai et al., 2010; Tsuji et al., 2012a,b; Lee E. B. et al., 2017; Porta et al., 2018) and hippocampus (Neumann et al., 2006) |
| Type B | Brain | + | • CTFs detected in cortex (Igaz et al., 2008; Arai et al., 2010; Tsuji et al., 2012a,b; Lee E. B. et al., 2017; Porta et al., 2018) | |
| Type C | Brain | + | • CTFs detected in cortex (Neumann et al., 2006, 2007; Igaz et al., 2008; Arai et al., 2010; Hasegawa et al., 2011; Tsuji et al., 2012a,b; Lee E. B. et al., 2017; Watanabe et al., 2018; Porta et al., 2018), hippocampus (Neumann et al., 2006; Tsuji et al., 2012a) and striatum (Tsuji et al., 2012a) | |
| - | • No CTFs in cerebellum, substantia nigra, pons, medulla or thalamus (Tsuji et al., 2012a) | |||
| Type D | Brain | ? | • Low levels of CTFs variably detected in frontal and temporal cortices (Neumann et al., 2007) | |
| Type E | Brain | + | • Multiple CTFs between 23 and 26 kDa detected in frontal cortex (Lee E. B. et al., 2017) | |
“+” indicates overt presence, “-” indicates no presence and “?’ indicates very low level detection or unclear findings.