Table 1.
Overview cell line characteristics.
| Original name | Alias | Mutation | Exonic genotype | Intronic HRE genotype | Source | Gender | Year of birth | Age at biopsy (years) | Reference (first published) |
|---|---|---|---|---|---|---|---|---|---|
| T12.9 | Ctrl1 | Control, parental to KO line below | WT/WT | WT/WT | Dresden (Sterneckert) | F | 1959 | N/A | Reinhardt et al (2013) |
| T12.9 KO | WT-KO | KO of exonic C9ORF72 in T12.9 (isogenic) | −/− | WT/WT | Dresden (Sterneckert) | F | 1959 | N/A | Abo-Rady et al (2020) |
| AKC5 | Ctrl2 | Control | WT/WT | WT/WT | Dresden (Hermann) | F | 1963 | 48 | Reinhardt et al (2013) |
| 30.1 | Ctrl3 | Control | WT/WT | WT/WT | Dresden (Sterneckert) | M | 1971 | N/A | Reinhardt et al (2013) |
| Pooled Ctrls | Ctrl | Pooled T12.9, AKC5, 30.1 | WT/WT | WT/WT | N/A | N/A | N/A | N/A | N/A |
| FUS-WT-EGFP C4 | FUS GC | WT FUS-EGFP (isogenic) | FUS-WT-EGFP/WT | WT/WT | Dresden (Hermann) | F | 1952 | 58 | Naumann et al (2018) |
| FUS-P525L-EGFP C21 | FUS | P525L FUS-EGFP (isogenic) | FUS-P525L-EGFP/WT | WT/WT | Dresden (Hermann) | F | 1952 | 58 | Naumann et al (2018) |
| TDP43 S393L | N/A | TDP43 S393L | TDP43/WT | WT/WT | Dresden (Hermann) | F | ND | 87 | Kreiter et al (2018) |
| TDP43 G294V | N/A | TDP43 G294V | TDP43/WT | WT/WT | Dresden (Hermann) | M | ND | 46 | Kreiter et al (2018) |
| Pooled TDP43 | TDP43 | Pooled TDP43 S393L and G294V | TDP43/WT | WT/WT | N/A | N/A | N/A | N/A | N/A |
| KDC28 | C9-1 | C9ORF-HRE (>50 rep.) | WT/WT | C9ORF-HRE/WT | Dresden (Hermann) | F | 1944 | 68 | Sivadasan et al (2016) |
| MHC30 | C9-2 | C9ORF-HRE (ca. 730 rep.) | WT/WT | C9ORF-HRE/WT | Dresden (Hermann) | F | 1961 | 51 | Sivadasan et al (2016) |
| 34.1 | C9-3 | C9ORF-HRE (>850) | WT/WT | C9ORF-HRE/WT | Dresden (Sterneckert) | Donnelly et al (2013) | |||
| JBR | C9-4 | C9ORF-HRE (ca. 1800 rep.) | WT/WT | C9ORF-HRE/WT | Ulm (Böckers, Ludolph, Demestre) | M | 1951 | NA | Higelin et al (2018), Catanese et al (2019) |
| 33.1 | C9 | C9ORF-HRE (>620 rep.), parental to isogenic lines below | WT/WT | C9ORF-HRE/WT | Dresden (Sterneckert) | M | 1944 | 65 | Donnelly et al (2013) |
| 33.1 GC | C9-GC | Gene-corrected 33.1 (isogenic) | WT/WT | WT/WT | Dresden (Sterneckert) | M | 1944 | 65 | Abo-Rady et al (2020) |
| 33.1 KO | C9-KO | KO of exonic C9ORF72 in 33.1 with preserved intronic HREs (isogenic) | −/− | C9ORF-HRE/WT | Dresden (Sterneckert) | M | 1944 | 65 | Abo-Rady et al (2020) |
| Pooled C9ORFs | C9ORF | Pooled KDC28, JBR, MHC30, 34.1, 33.1 | WT/WT | C9ORF-HRE/WT | N/A | N/A | N/A | N/A | N/A |