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.