Table 1.

Overview cell line characteristics.

Original nameAliasMutationExonic genotypeIntronic HRE genotypeSourceGenderYear of birthAge at biopsy (years)Reference (first published)
T12.9Ctrl1Control, parental to KO line belowWT/WTWT/WTDresden (Sterneckert)F1959N/AReinhardt et al (2013)
T12.9 KOWT-KOKO of exonic C9ORF72 in T12.9 (isogenic)−/−WT/WTDresden (Sterneckert)F1959N/AAbo-Rady et al (2020)
AKC5Ctrl2ControlWT/WTWT/WTDresden (Hermann)F196348Reinhardt et al (2013)
30.1Ctrl3ControlWT/WTWT/WTDresden (Sterneckert)M1971N/AReinhardt et al (2013)
Pooled CtrlsCtrlPooled T12.9, AKC5, 30.1WT/WTWT/WTN/AN/AN/AN/AN/A
FUS-WT-EGFP C4FUS GCWT FUS-EGFP (isogenic)FUS-WT-EGFP/WTWT/WTDresden (Hermann)F195258Naumann et al (2018)
FUS-P525L-EGFP C21FUSP525L FUS-EGFP (isogenic)FUS-P525L-EGFP/WTWT/WTDresden (Hermann)F195258Naumann et al (2018)
TDP43 S393LN/ATDP43 S393LTDP43/WTWT/WTDresden (Hermann)FND87Kreiter et al (2018)
TDP43 G294VN/ATDP43 G294VTDP43/WTWT/WTDresden (Hermann)MND46Kreiter et al (2018)
Pooled TDP43TDP43Pooled TDP43 S393L and G294VTDP43/WTWT/WTN/AN/AN/AN/AN/A
KDC28C9-1C9ORF-HRE (>50 rep.)WT/WTC9ORF-HRE/WTDresden (Hermann)F194468Sivadasan et al (2016)
MHC30C9-2C9ORF-HRE (ca. 730 rep.)WT/WTC9ORF-HRE/WTDresden (Hermann)F196151Sivadasan et al (2016)
34.1C9-3C9ORF-HRE (>850)WT/WTC9ORF-HRE/WTDresden (Sterneckert)Donnelly et al (2013)
JBRC9-4C9ORF-HRE (ca. 1800 rep.)WT/WTC9ORF-HRE/WTUlm (Böckers, Ludolph, Demestre)M1951NAHigelin et al (2018), Catanese et al (2019)
33.1C9C9ORF-HRE (>620 rep.), parental to isogenic lines belowWT/WTC9ORF-HRE/WTDresden (Sterneckert)M194465Donnelly et al (2013)
33.1 GCC9-GCGene-corrected 33.1 (isogenic)WT/WTWT/WTDresden (Sterneckert)M194465Abo-Rady et al (2020)
33.1 KOC9-KOKO of exonic C9ORF72 in 33.1 with preserved intronic HREs (isogenic)−/−C9ORF-HRE/WTDresden (Sterneckert)M194465Abo-Rady et al (2020)
Pooled C9ORFsC9ORFPooled KDC28, JBR, MHC30, 34.1, 33.1WT/WTC9ORF-HRE/WTN/AN/AN/AN/AN/A