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. 2018 Oct 30;11:394. doi: 10.3389/fnmol.2018.00394

Table 2.

Published ALS yeast models and their characteristics.

Human ALS protein (wild-type and mutants) Toxicity Aggregation Reference
TDP-43 G294A, Q331K, M337V, Q343R, N345K, R361S, N390D Yes Yes Johnson et al. (2009), Armakola et al. (2011), Braun et al. (2011), Kryndushkin and Shewmaker (2011), Sun et al. (2011), Jackrel et al. (2014), Liu et al. (2017) and Leibiger et al. (2018)
FUS R524S, P525L Yes Yes Fushimi et al. (2011), Ju et al. (2011), Kryndushkin and Shewmaker (2011), Kryndushkin et al. (2011), Sun et al. (2011), Daigle et al. (2013) and Jackrel et al. (2014)
SOD1 A3V, G36R, H47Q, G92A, S133N No No Nishida et al. (1994), Rabizadeh et al. (1995), Corson et al. (1998), Gunther et al. (2004), Bastow et al. (2011) and Bastow et al. (2016)
C9orf72 (GA)50, (GR)100, (PA)50, (PR)50 Yes Not assessed Jovičić et al. (2015) and Chai and Gitler (2018)
Ataxin-2 Q22, Q79 No Not assessed Ralser et al. (2005), Nonhoff et al. (2007), Elden et al. (2010) and Bonini and Gitler (2011)
OPTN E50K, E478G Yes Yes Kryndushkin et al. (2012)
PFN1 C71G, T109M, M114T, E117G, G118V, R136W, H120E No No assessed Figley et al. (2014)
hnRNPA1, hnRNPA2B1 hnRNPA1 D262V, hnRNPA1 D262N, hnRNPA2B1 D290V Yes Yes Kim et al. (2013)
VAPB P56S Wild-type—Yes P56S—not assessed Yes Suzuki et al. (2009), Nakamichi et al. (2011)
VCP R155C, A232E, T761E, K524A No Wild-type, T761E, K524A—No R155C and A232E—Yes Takata et al. (2012)
SETX No No Richard et al. (2013), Bennett and La Spada (2018)
ANG Yes Yes Jo et al. (2017)
TAF 15 Yes Yes Couthouis et al. (2011)
UBQLN2 (essential domain only) No Not assessed Gilpin et al. (2015)

Wild-type and ALS-associated mutant proteins that have been expressed in yeast are listed and categorized based on toxicity and aggregation phenotypes. Toxicity refers to growth defects of yeast cells expressing ALS proteins. Aggregation refers to fluorescent microscopically assessed inclusions or foci. Note, that even though there is a strong correlated between aggregation and toxicity of ALS proteins in yeast, this does not equate with a causative relation.