ATP	adenosine triphosphate
AAV	adeno-associated virus
AD	Alzheimer’s disease
adPEO	autosomal dominant PEO
AHS	Alpers–Huttenlocher syndrome
ANS	ataxia neuropathy spectrum
arPEO	autosomal recessive PEO
ASO	antisense RNA oligonucleotide
cap	capsid
COX	cytochrome oxidase
CPEO	chronic PEO
CPEO	chronic progressive external ophthalmoplegia
CRISPR	clustered regularly interspaced short palindromic repeats
CSBI	conserved sequence block I
DdCBE	DddA-derived cytosine base editor
DddA	double-stranded deaminase A
DEAF	deafness or aminoglycoside-induced deafness
DF-MITO-Porter	dual function MITO-Porter
DOPE	1,2-dioleoyl-sn-glycero-3-phosphatidylethanolamine
DQAplexes	DQAsome-DNA complexes
ELAC2	elaC ribonuclease Z 2
GAPDH	glyceraldehyde 3-phosphate dehydrogenase
gRNA	guide RNA
HLV	hydrodynamic limb vein
HSP1	heavy strand promoter 1
ITRs	inverted terminal repeats
LHON	Leber’s hereditary optic neuropathy
LSP	light strand promoter
MCHS	myocerebrohepatopathy spectrum
MELAS	mitochondrial encephalopathy, lactic acidosis, stroke-like episodes
MEMSA	myopathy sensory ataxia
MERRF	myoclonic epilepsy with ragged red fibers
MI	myocardial infarction
MILS	maternally inherited Leigh syndrome
MIRAS	Mitochondrial recessive ataxia syndrome
MIRAS	mitochondrial recessive ataxia syndrome
mRNAs	messenger RNAs
MRP	mitochondrial RNA processing
MRPS12	mitochondrial ribosomal protein S12
mtDNA	mitochondrial DNA
MTS	mitochondrial targeting sequence (
mtTALENs	mitochondria-targeted TALE nucleases
mtZFN	mitochondria-targeted zinc-finger nuclease
NARP	neurogenic muscle weakness, ataxia, and retinitis pigmentosa
nDNA	nuclear DNA
nt	nucleotides
OH	origin of the H-strand
PEI	polyethyleneimine
PEO	progressive external ophthalmoplegia
PNAs	peptide nucleic acids
PNPASE	polynucleotide phosphorylase
POLRMT	mitochondrial RNA polymerase
POLγ	DNA polymerase gamma
rep	replicase
RMRP	RNase MRP
rRNAs	ribosomal RNAs
SSBP1	single-stranded DNA-binding protein
TALEs	transcription activator-like effectors
TEFM	mitochondrial transcription elongation factor
TFAM	mitochondrial transcription factor A
TFB2M	mitochondrial transcription factor B2
TPA	tetraphenylarsonium
TPMP	triphenylmethylphosphonium
TPP	tetraphenylphosphonium
tRNAs	transfer RNAs
UTR	untranslated region