ALS	Amyotrophic Lateral Sclerosis
APE1	Apurinic/Apyrimidinic Endonuclease 1
ATF4	Activating Transcription Factor 4
ATF5	Activating Transcription Factor 5
BER	Base Excision Repair pathway
C9ORF72	Chromosome 9 Open Reading Frame 72
CCCP	Carbonyl Cyanide m-Chlorophenylhydrazone
CHOP	C/EBP Homologous Protein (DDIT3)
CLPP	Caseinolytic mitochondrial matrix peptidase proteolytic subunit
DMEM	Dulbecco’s Modified Eagle Medium
DNA polymerase γ	Mitochondrial DNA polymerase gamma
eIF2α	Eukaryotic Initiation Factor 2 alpha
ER	Endoplasmic Reticulum
ETC	Electron Transport Chain
FBS	Fetal Bovine Serum
FOXO3A	Forkhead Box O3
GFP	Green Fluorescent Protein
LC3	Microtubule-associated protein 1 light chain 3
LONP1	Lon Peptidase 1 Mitochondrial
MIC10	MICOS Complex Subunit MIC10 (C1ORF151)
MIC60	MICOS Complex Subunit MIC60 (IMMT)
MICOS	Mitochondrial Contact Site and Cristae Organizing System
MQC	Mitochondrial Quality Control
mtDNA	Mitochondrial DNA
OGG1	8-Oxoguanine DNA Glycosylase 1
PBS	Phosphate-Buffered Saline
PERK	Protein kinase R (PKR)-like endoplasmic reticulum kinase
PINK1	PTEN-induced kinase 1
qPCR	Quantitative Polymerase Chain Reaction
RIPA	Radioimmunoprecipitation Assay buffer
ROIs	Regions of Interest
ROS	Reactive Oxygen Species
SDS-PAGE	Sodium Dodecyl Sulfate–Polyacrylamide Gel Electrophoresis
SIRT3	Sirtuin 3
SOD1	Superoxide Dismutase 1.
TARDBP	TAR DNA Binding Protein gene
TBS-T	Tris-Buffered Saline with Tween 20
TDP-43	TAR DNA-binding protein 43
TEM	Transmission Electron Microscopy
TOM20	Translocase of Outer Mitochondrial Membrane 20
TRAP1	TNF Receptor Associated Protein 1
UPRER	Endoplasmic Reticulum Unfolded Protein Response
UPRmt	Mitochondrial Unfolded Protein Response