Table 1.
Strain, phenotype, and availability information for C. elegans neurodegenerative disease models.
| NDs | Model | Strain name(s) | Expression in C. elegans |
Phenotypes | References | CGC? |
|---|---|---|---|---|---|---|
| polyQ | Is[unc-54::polyQ(19, 82)::gfp/yfp/cfp] | n.a.* | Constitutive muscle | Length-dependent aggregate formation; development delays, motility defects. | (Satyal et al., 2000) | No |
| rmIs[unc-54::polyQ(0, 19, 29, 33, 35, 37, 40, 44, 64, 82)::yfp] | AM140, 141, 470 | Constitutive muscle | Stark aggregation threshold at Q35-40 accompanied by motility defects; Threshold is dynamic and influenced by animal longevity. | (Morley et al., 2002) | No | |
| rmEx[rgef-1::polyQ(0, 19, 35, 40, 67, 86)::cfp/yfp] | AM78, 80, 81, 83, 85, 87; AM303, 305, 308, 313, 322, 324 | Constitutive pan-neuronal | PolyQ length-dependent aggregation and neuronal dysfunction: dysfunctional thrashing, pharyngeal pumping; erratic defecation cycle. | (Brignull et al., 2006) | Yes | |
| HD | rtIs11[osm-10::gfp + osm-10::htt(Q2, 23, 95, 150) + dpy-20(+)] | n.a. | Chemosensory neurons | Q150 expression induces ASH neuronal degeneration, but not death. | (Faber et al., 1999) | Yes |
| Is[mec-3::htt57(Q19, 88, 128)::gfp; mec-3::htt57(Q19, 88, 128)::cfp + mec-3::yfp] | ID1, ID24 | Mechanosensory neurons | Substantial posterior touch insensitivity and anterior Mec phenotype. Significant aggregate deposition and PLM axon abnormalities. | (Parker et al., 2001) | No | |
| rtIs11[osm-10::gfp + osm-10::htt(Q150) + dpy-20(+)] | HA659 | Chemosensory neurons | Genetic screen identified polyQ enhancer-1 (PQE-1); Loss of PQE-1 strongly enhanced neurodegeneration, while overexpression has protective effect | (Faber et al., 2002) | Yes | |
| Is[unc-54::htt513(Q15, Q128)::yfp] | EAK102, 103 | Constitutive muscle | Length-dependent aggregation and toxicity; Htt513(Q128) animals show motor dysfunction and modest decrease in longevity. | (Lee et al., 2017) | Yes | |
| MJD | Full-length ATXN-3 lines: Is[rgef-1::(AT3q14, AT3q75, AT3q130)::yfp] | AM491, 494, 509, 513, 519, 520, 599, 666, 685 | Constitutive pan-neuronal | PolyQ length-dependent aggregation, motor dysfunction; Ventral and dorsal nerve cord neurons highly affected. | (Teixeira-Castro et al., 2011) | No |
| C-terminal ATXN-3 lines: Is[rgef-1::(257cAT3q14, 257cAT3q75, 257cAT3q80, 257cAT3q128)::yfp] | AM391, 396, 416, 419, 420, 422, 428, 683, 684, 702 | Constitutive pan-neuronal | Similar aggregation profile to full-length ATXN-3 with more severe motor phenotype. | (Teixeira-Castro et al., 2011) | No | |
| Is[unc-54::257cAT3(Q45, Q63)::yfp] | n.a. | Constitutive muscle | PolyQ length-dependent aggregation and toxicity not significantly affected by aging. | (Christie et al., 2014) | No | |
| AD | Is[unc-54::Aβ1–42] (WT) or dimer Is[Aβ1–42] | CL1019, 1118, 1119, 1120, 1121, 2005, 2006, 2109, 2120, 3109 | Constitutive muscle | Progressive age-dependent paralysis with formation of amyloid deposits. | (Link, C.D., 1995) | Yes |
| Is[unc-54::Met35Cys Aβ1–42] | CL3115 | Constitutive muscle | No formation of amyloid deposits and no increases in oxidative stress. | (Yatin et al., 1999) | No | |
| smg-1(cc546); Is[snb-1::Aβ1–42 + mtl-2::gfp] | CL2241, 2355 | Inducible pan-neuronal | Defective chemotaxis, formation of amyloid deposits, and serotonin hypersensitivity. | (Wu et al., 2006) | Yes | |
| 3::Aβ1–42::let UTR) + (rol-6(su1006)] | CL4176 | Inducible body-wall muscle | Rapid paralysis; oxidative stress which precedes Aβ fibril formation. | (Drake et al., 2003) | Yes | |
| dvIs100[CL354(unc-54::DA-Aβ1–42) + CL26(mtl-2::gfp)] | GMC101 | Constitutive muscle | Severe paralysis following 48hr exposure to 25°C temperature shift. | (McColl et al., 2012) | Yes | |
| Is[eat-4::ssAβ1–42(N-term) + eat-4::gfp + myo-2::mCherry] | UA166 | Glutamatergic neurons | Age-dependent loss of glutamatergic neurons. | (Treusch et al., 2011) | No | |
| Tauopathies/FTDP-17 | Is[aex-3::4R1N hTau (WT, V337M, P301L) + myo-2::gfp] | CK10, 49, 1301, 1310 | Constitutive pan-neuronal | V337M and P301L mutants show age-dependent uncoordinated phenotype, insoluble tau fibrils, and reduced lifespan. | (Kraemer et al., 2003) | No |
| tmIs[mec-7::tau WT(0N4R, 0N3R) + rol-6(su1006)] | n.a. | Mechanosensory neurons | Mild progressive impairment in touch response; Little detectable tau accumulation in PLM tail neuron. | (Miyasaka et al., 2005) | No | |
| tmIs[mec-7::tau(P301L, R406W) + rol-6(su1006)] | n.a. | Mechanosensory neurons | Severe progressive impairment in touch response, significant tau accumulation in PLM tail neuron. | (Miyasaka et al., 2005) | No | |
| pha-1(e2123ts); Ex[rgef-1::Tau352(WT, PHP, Ala10) + pha-1(+)] | VH254, 255, 418, 421, 1014, 1015, 1016, 1018 | Constitutive pan-neuronal | Progressive motor dysfunction and neurodegeneration. PHP tau induces abnormal motor neuron development; Ala10 worms show early motor impairments and shortened lifespan. | (Brandt et al., 2009) | Yes | |
| Pro-aggregant lines: Is[rab-3::F3ΔK280 + myo-2::mCherry] | BR5270, 5485, 5706, 5944 | Constitutive pan-neuronal | Severe locomotive impairment at day 1 of adulthood; Accelerated aggregate formation; Severe developmental defects in nervous system; impairments in presynaptic transmission. | (Fatouros et al., 2012) | Yes | |
| Anti-aggregant lines: Is[rab-3::F3ΔK280(I277P)(I308P) + myo-2::mCherry] | BR5271, 5486, 6427, 6516 | Constitutive pan-neuronal | No overt locomotive impairment; minimal effects on neurodevelopment. | (Fatouros et al., 2012) | Yes | |
| ALS | ALS Is[hsp-16.2::sod-1 (WT, A4V, G37R, G93A) + myo-3::sod-1(WT, A4V)::gfp + rol-6(su1006)] | n.a. | Heat-shock inducible muscles | Oxidative stress-induced aggregate formation. | (Oeda et al., 2001) | No |
| Is[snb-1::sod-1(WT, G85R)::yfp] | n.a. | Constitutive pan-neuronal | G85R mutants exhibit severe motor dysfunction accompanied by both soluble oligomers and insoluble aggregate deposits. | (Wang et al., 2009) | No | |
| Is[sng-1::sod-1(WT, A4V, G37R, G93C)::gfp] | n.a. | Constitutive pan-neuronal | Mutant homodimers show increased aggregate formation compared to heterodimers, but G85R heterodimers are more toxic in functional assays. | (Witan et al., 2008) | No | |
| Is[unc-54::sod-1(WT, G85R, G93A, G127insTGGGstop)::yfp] | AM263, 265 | Constitutive muscle | SOD-1 mutants show morphologically heterogenous aggregates with varied biophysical properties and mild motility defects | (Gidalevitz et al., 2009) | Yes | |
| Is[unc-25::sod-1(G93A)::gfp] | n.a. | GABAergic motor neurons | G93A SOD-1 animals show progressive motor dysfunction, aggregate formation, and axonal guidance defects. | (Li et al., 2013) | No | |
| lin-15(n765ts); [rgef-1::fus(WT, R514G, R521G, R522G, R524S, P525L) + pab-1::mCherry; lin-15(+)] | n.a. | Constitutive pan-neuronal | Cytoplasmic FUS aggregates; R522G, P525L, FUS513 and FUS501: reduced lifespan. P525L, FUS513 and FUS501: partial or complete paralysis. | (Murakami et al., 2012) | No | |
| Is[snb-1::tdp-43 (WT, G290A, A315T, M337V) + snb-1::gfp] | CK405, 406, 410, 422, 423, 426 | Constitutive pan-neuronal | TDP-43 mutants exhibited severe motor dysfunction. Motor neuron degeneration accompanied by hyperphosphorylation, ubiquitination, and truncated TDP-43 in aggregates. | (Liachko et al., 2010) | No | |
| Is[unc-25::snb-1::gfp] + Ex[snb-1::tdp-43; regf-1::DsRed2; unc122::rfp] | CL1681, 1682, 2609 | Constitutive pan-neuronal | Uncoordinated motor phenotype; abnormal motor neuron synapses. | (Ash et al., 2010) | No | |
| PD | Is[dat-1::α-syn (WT, A53T) + dat-1::gfp] | BY273; UA18, 31, 44 | Dopaminergic neurons | Neuronal loss and dendritic breaks shown in both WT and A53T animals. | (Lakso et al., 2003) | No |
| Is[unc-54::α-syn::yfp + unc-119(+)] | NL5901 | Constitutive muscle | Age-dependent inclusion formation. | (van Ham et al., 2008) | Yes | |
| Is[unc-54::α-syn::gfp + rol-6(su1006)] | UA49 | Constitutive muscle | Misfolding and accumulation of α-synuclein aggregates. | (Hamamichi et al., 2008) | No | |
| Is[unc-51::α-syn(WT, A53T, A30P) + unc-51::egfp] | n.a. | Constitutive pan-neuronal | No motor dysfunction or developmental defects; RNAi knock-down of AP-2 complex subunits induced severe growth/motor abnormalities. | (Kuwahara et al., 2008) | No | |
| Is[mec-7::α-syn(WT, A53T) + rol-6(su1006)] | n.a. | Mechanosensory neurons | Moderate impairments in touch response, impaired neuromuscular transmission. | (Kuwahara et al., 2008) | No | |
| Is[dat-1::α-syn(A30P, A53T, A56P) + dat- 1::mCherry] | n.a. | Dopaminergic neurons | A56P and TP: Pronounced neurodegeneration, DA loss, and significant impairment in DA-dependent behavior; A30P and A53T: Moderate decrease in DA levels, DA-dependent behavioral impairments. | (Karpinar et al., 2009) | No | |
| unc-51::α-syn(S129A, S129D) + unc-51::egfp | n.a. | Constitutive pan-neuronal | Severe motor dysfunction beginning in early development, stunted growth, and synaptic abnormalities. | (Kuwahara et al., 2012) | No | |
| lin-15(n765ts); wlzIs1-7[snb-1::lrrk2(WT, G2019S, R1441C, KD, R1441C/KD) + mec-4::gfp; lin-15(+)] | n.a. | Constitutive pan-neuronal | WT LRRK2 protects against mitochondrial dysfunction. G2019S mutants increased vulnerability of DA neurons to mitochondrial stress. | (Saha et al., 2009) | Yes | |
| baIn20[dat-1::lrrk2(G2019S) + dat-1::gfp] | UA118 | Dopaminergic neurons | Progressive degeneration of DA neurons, significant depletion of dopamine accompanied by locomotor dysfunction and behavioral deficits. G2019S mutants have more rapid progression of disease phenotype. | (Liu et al., 2011) | No | |
| BY250; baEx129[dat-1::lrrk2(G2019S/D1994A)] | UA215, 216 | Dopaminergic neurons | Progressive degeneration of DA neurons accompanied by behavioral deficits, motor dysfunction, and DA depletion; G2019S strain more severe. | (Liu et al., 2011) | No | |
| lin-15(n765ts)X; Is[dat-1::lrrk2(WT, R1441C, G2019S, K1347A) + dat-1::gfp + lin-15(+)] | SGC722, 851, 856, 862 | Dopaminergic neurons | Progressive degeneration of DA neurons and DA loss; behavioral and motor deficits. | (Yao et al., 2013) | No | |
| lin-15(n765ts)X; cwrEx900[dat-1::gfp, dat-1::lrrk2(R1441C/A2016T), lin-15(+)] | SG900, 910 | Dopaminergic neurons | Double mutants show similar profile to R1441C and G2019S LRRK2 models; DAergic defects and neurodegeneration. | (Yao et al., 2013) | No | |
| Prion | cgIs51-53[ric-19::prp + ric-19::gfp] | n.a. | Constitutive pan-neuronal | High PrP levels alter morphology; significant motor impairment and reduction in lifespan. | (Park and Li, 2011) | No |
| lin-15(n765ts); Is[mec-7::prp(WT, PG13) + str-1::gfp; mec-7::gfp + lin-15(+)] | n.a. | Mechanosensory neurons | PG13-PrP mutants show progressive loss of tail-touch response without neuronal death. | (Bizat et al., 2010) | No | |
| rmIs319[unc-54::sup35(RΔ2—5, NM, R2E2)::yfp] | AM801, 803, 806 | Constitutive muscle | Severe disruption of mitochondrial integrity, embryonic/larval arrest, developmental delays, tissue defects, loss of proteostasis. | (Nussbaum-Krammer et al., 2013) | No |
Genetic nomenclature of C. elegans ND models, expression pattern, observable phenotypes, and availability of strain(s) through the Caenorhabditis Genetics Center (CGC).
*
Denotes that strain nomenclature was not provided by the creators of the model(s) in the primary literature.