Table 1.

Summary of prion‐like evidence for FTD‐associated proteins in vitro, in vivo, and in patients

Protein	Human disease	Subcellular localization of aggregates	Seeded aggregation			Cell‐to‐cell spread		Experimentally transmitted disease	Strains	Pathology Staging in humans
			In vitro	In cell culture	In vivo	In cell culture	In vivo
Tau	AD, FTLD‐Tau (FTLD‐17 MAPT, PSP, AGD, PiD, CBD), CTE	Cytoplasmic	Friedhoff et al. 1998;	Frost et al. 2009; Guo and Lee 2011; Wu et al. 2013; Nonaka et al. 2010;	Bolmont 2007, Clavaguera et al. 2009, 2013; Iba et al. 2013; Peeraer et al. 2015; Lasagna‐Reeves et al. 2012;	Kfoury et al. 2012; Iba et al. 2013;	Lasagna‐Reeves et al. 2012; de Calignon et al. 2012; Liu et al. 2012; Iba et al. 2013, Ahmed et al. 2014; Dujardin et al. 2014;	Peeraer et al. 2015;	Sanders et al. 2014; Boluda et al. 2015	AD (Braak and Braak 1991; Braak and Braak 1995; Braak et al. 2006), CTE (Geddes et al. 1999; McKee et al. 2013)
TDP‐43	FTLD‐TDP, ALS	Mostly cytoplasmic	Furukawa et al. 2011;	Furukawa et al. 2011; Feiler et al. 2015;	n.d.	Nonaka et al. 2013; Feiler et al. 2015;	n.d.	n.d.		FTLD (Brettschneider et al. 2014), ALS (Brettschneider et al. 2013)
FUS	FTLD‐FUS, ALS	Mostly cytoplasmic, and rare intranuclear	Nomura et al. 2014	n.d.	n.d.	n.d.	n.d.	n.d.	n.d.	n.d.

AD, Alzheimer disease; AGD, argyrophilic grain disease; ALS, amyotrophic lateral sclerosis; CBD, corticobasal degeneration; CTE, chronic traumatic encephalopathy; FTD, frontotemporal dementia; FTLD, frontotemporal lobar degeneration; FUS, Fused in sarcoma; PiD, Pick's disease; PSP, progressive supranuclear palsy; TDP‐43, TAR DNA‐binding protein of 43 kDa; n.d., not determined.