Table 1.

TDP-43 cleavage sites and responsible enzymes

Enzyme	Cleavage site	Fragment size (kDa) and type	Experimental system	References
Caspase 3	89, 219, 247	25, 35, 42 CTF	Cell culture	(Zhang et al., 2007)
Caspase 4	174	25 CTF	in vitro and in monkey	(Li et al., 2015)
Caspase 7	89, 219	25, 35, 42 CTF	Cell culture	(Zhang et al., 2007)
Caspase (unknown)	169	27 CTF	Cell culture	(Suzuki et al., 2011)
Calpain 1 and Calpain 2	229, 243, 246, 286, 295, 297, 318, 324	32, 33, 36 CTF and NTF	Cell culture, mouse and human ALS specimens	(Yamashita et al., 2012; Yang et al., 2014b)
Asparaginyl endopeptidase (AEP)	70, 76, 279, 291, 285, 306, 372	22-37 CTF	Cell culture and Mouse	(Herskowitz et al., 2012)
Undetermined	55, 108, 208, 130, 164, 214, 266, 400	15-43 NTF and CTF	Mouse	(Igaz et al., 2008; Kametani et al., 2016)