Table 2.

List of abbreviations in alphabetical order.

AbbreviationAliasFull term
53BP1Tumor suppressor 53–binding protein 1 and DSB marker
ALSAbramyotrophic lateral sclerosis
ASOAntisense oligonucleotide
ATGAdenine–thymine–guanine start codon of translation
BNS-22DNA topoisomerase 2 inhibition without causing SSBs or DSBs
C9C9ORF, C9ORF72Chromosome 9 open reading frame 72
C9-GCGene-corrected C9 isogenic cell line, that is, with HREs in intron 1 of C9ORF72 gene excised
C9-KOIsogenic C9 cell line with functional KO of the exonic, ATG-transcribed C9ORF72 gene part with HREs in intron 1 preserved
CamptoCamptothecin, DNA topoisomerase 1 inhibitor, and SSB inducer
Cas9nCRISPR-associated 9 nickase
Casp3Apoptosis marker cleaved caspase 3
CRISPRClustered regularly interspaced short palindromic repeats
CtrlWild-type control cell line
De.g. D14, D21, D80Days after seeding of MNs into MFCs
DEGDifferentially expressed gene
DMSODimethylsulfoxide, common solvent for chemcial compounds, inhibitors, etc.
DPRDipeptide repeat protein, encoded by RAN translation of intronic HREs
DSBDNA double-strand break
ELISAEnzyme-linked immunosorbent assay
EtoEtoposide, DNA topoisomerase 2 inhibitor, and DSB inducer
FISHFluorescence in situ hybridization
FUSFused in sarcoma
GGGGCCGuanine(x4)-cytosine(x2) hexanucleotide sequence motif of HREs
GOFGain of (toxic) function
HCHigh content
hnRNPHeterogeneous ribonucleoprotein
HREHexanucleotide repeat expansion in intron 1 of C9ORF72 gene
HSPHeat shock protein
iPSCInduced pluripotent stem cell
KDGene knockdown
KOGene knockout
LNALocked nucleic acid nucleotides, used in RNA FISH probes
LOFLoss of function
LUTLook up table
MAP2Microtubule-associated protein 2 and dendritic neuron marker
MFCXona® microfluidic chamber, for compartmentalized neuron cultures with axons aligned in microchannels with defined orientation with respect to somata and clear physical separation from dendrites
MNMotoneuron
nHRNormal hexanucleotide repeats
NPCNeuronal progenitor cell
OXPHOSOxidative phosphorylation, referring to the mitochondrial respiratory ATP production
pATMPhosphorylated ataxia telangiectasia mutated protein
poly GAGAPoly glycine–alanine DPR, one species in the spectrum of RAN-translated HREs
poly GPGPPoly glycine–proline DPR, one species in the spectrum of RAN-translated HREs
poly PRPRPoly proline–arginine DPR, one species in the spectrum of RAN-translated HREs
PPIProtein–protein interaction
RAN translationRepeat-associated non-ATG translation
R-loopsThree-stranded hybrid structure composed of DNA template and complementary strand and nascent mRNA during transcription
ROSReactive oxygen species
RPS25Ribosomal protein subunit of 25 kDa
RRERNA repeat expansion
SSBDNA single-strand break
TDP43TAR DNA-binding protein-43
WTWild type
γH2AXPhosphorylated histone H2A.X, DSB marker