List of abbreviations in alphabetical order.
| Abbreviation | Alias | Full term |
|---|---|---|
| 53BP1 | Tumor suppressor 53–binding protein 1 and DSB marker | |
| ALS | Abramyotrophic lateral sclerosis | |
| ASO | Antisense oligonucleotide | |
| ATG | Adenine–thymine–guanine start codon of translation | |
| BNS-22 | DNA topoisomerase 2 inhibition without causing SSBs or DSBs | |
| C9 | C9ORF, C9ORF72 | Chromosome 9 open reading frame 72 |
| C9-GC | Gene-corrected C9 isogenic cell line, that is, with HREs in intron 1 of C9ORF72 gene excised | |
| C9-KO | Isogenic C9 cell line with functional KO of the exonic, ATG-transcribed C9ORF72 gene part with HREs in intron 1 preserved | |
| Campto | Camptothecin, DNA topoisomerase 1 inhibitor, and SSB inducer | |
| Cas9n | CRISPR-associated 9 nickase | |
| Casp3 | Apoptosis marker cleaved caspase 3 | |
| CRISPR | Clustered regularly interspaced short palindromic repeats | |
| Ctrl | Wild-type control cell line | |
| D | e.g. D14, D21, D80 | Days after seeding of MNs into MFCs |
| DEG | Differentially expressed gene | |
| DMSO | Dimethylsulfoxide, common solvent for chemcial compounds, inhibitors, etc. | |
| DPR | Dipeptide repeat protein, encoded by RAN translation of intronic HREs | |
| DSB | DNA double-strand break | |
| ELISA | Enzyme-linked immunosorbent assay | |
| Eto | Etoposide, DNA topoisomerase 2 inhibitor, and DSB inducer | |
| FISH | Fluorescence in situ hybridization | |
| FUS | Fused in sarcoma | |
| GGGGCC | Guanine(x4)-cytosine(x2) hexanucleotide sequence motif of HREs | |
| GOF | Gain of (toxic) function | |
| HC | High content | |
| hnRNP | Heterogeneous ribonucleoprotein | |
| HRE | Hexanucleotide repeat expansion in intron 1 of C9ORF72 gene | |
| HSP | Heat shock protein | |
| iPSC | Induced pluripotent stem cell | |
| KD | Gene knockdown | |
| KO | Gene knockout | |
| LNA | Locked nucleic acid nucleotides, used in RNA FISH probes | |
| LOF | Loss of function | |
| LUT | Look up table | |
| MAP2 | Microtubule-associated protein 2 and dendritic neuron marker | |
| MFC | Xona® microfluidic chamber, for compartmentalized neuron cultures with axons aligned in microchannels with defined orientation with respect to somata and clear physical separation from dendrites | |
| MN | Motoneuron | |
| nHR | Normal hexanucleotide repeats | |
| NPC | Neuronal progenitor cell | |
| OXPHOS | Oxidative phosphorylation, referring to the mitochondrial respiratory ATP production | |
| pATM | Phosphorylated ataxia telangiectasia mutated protein | |
| poly GA | GA | Poly glycine–alanine DPR, one species in the spectrum of RAN-translated HREs |
| poly GP | GP | Poly glycine–proline DPR, one species in the spectrum of RAN-translated HREs |
| poly PR | PR | Poly proline–arginine DPR, one species in the spectrum of RAN-translated HREs |
| PPI | Protein–protein interaction | |
| RAN translation | Repeat-associated non-ATG translation | |
| R-loops | Three-stranded hybrid structure composed of DNA template and complementary strand and nascent mRNA during transcription | |
| ROS | Reactive oxygen species | |
| RPS25 | Ribosomal protein subunit of 25 kDa | |
| RRE | RNA repeat expansion | |
| SSB | DNA single-strand break | |
| TDP43 | TAR DNA-binding protein-43 | |
| WT | Wild type | |
| γH2AX | Phosphorylated histone H2A.X, DSB marker |