AAV	adeno-associated virus
Al	aluminium
AMPK	adenosine monophosphate-dependent protein kinase
ATF4	activating transcription factor
BCS	bathocuproine disulphonate
BiP	binding immunoglobulin protein
Cer	ceramide
Cnc	cap-n-collar
CNS	central nervous system
CRISPR	clustered, regularly interspaced, short palindromic repeat
Cu	copper
CyaY	Escherichia coli frataxin homolog
Da	daughterless
DamID	targeted DNA adenine methyltransferase identification
DFOB	desferrioxamine B
DFP	deferiprone
dhCer	dihydroceramide
dhSph	dihydrosphingosine
DMT1	divalent metal transporter-1
DN	dentate nucleus
DRG	dorsal root ganglia
DTT	dithiothreitol
eIF2α	α subunit of eukaryotic translation initiation factor 2
EMS	ethyl methanesulfonate
ER	endoplasmic reticulum
ETC	electron transport chain
Fe	iron
Fe–S	iron–sulfur
FFA	free fatty acid
fh	frataxin homolog
FLP/FRT	flippase and flippase recognition target
FRDA	Friedreich’s ataxia
FXN	human frataxin
GAA	guanine-adenine-adenine
GC/MS	gas chromatography coupled with mass spectrometry
Grx1	glutaredoxine
GSH	reduced glutathione
GSSG	oxidized glutathione
HIFs	hypoxia inducible factors
HRE	hypoxia responsive element
HSAN1	hereditary sensory and autonomic neuropathy type 1
IREs	iron responsive elements
IRP-1A	iron regulatory protein 1A
KO	knock-out
lace	palmitoyltransferase
MAMs	mitochondria-associated membranes
Marf	mitofusin
MCK	muscle creatine kinase
Mef-2	myocyte enhancer factor-2
Mn	manganese
MPP	mitochondrial processing peptidase
Mvl	Malvolio
PBA	4-phenylbutyric acid
Pdk1	protein kinase-1
PNS	peripheral nervous system
PPARγ	peroxisome proliferator-activated receptor gamma
RNAi	RNA interference
ROS	reactive oxygen species
SOD1	cytosolic superoxide dismutase
SOD2	mitochondrial superoxide dismutase
Sph	sphingosine
SREBP1	sterol-responsive element-binding protein 1
TPEN	N,N,N′,N′-tetrakis(2-pyridinylmethyl)-1,2-ethanediamine
Tsf	transferrins
TTM	tetrathiomolybdate
TUDCA	tauroursodeoxycholic acid
UAS	upstream activating sequence
Yfh1	yeast frataxin homolog 1
Zn	zinc