Acetyl-CoA	Acetyl coenzyme A
ATP	Adenosine triphosphate
ATXN3	Ataxin-3
ATG3	Ubiquitin-like-conjugating enzyme ATG3
BCA	Bicinchoninic acid assay
BSA	Bovine serum albumin
CII	Complex II
CIII	Complex III
CI	Complex I
CHCHD2	Coiled–coil–helix–coiled–coil–helix domain-containing protein 2
CHST3	Carbohydrate sulfotransferase 3
CoA-SH	Coenzyme A (thiol form)
CytC	Cytochrome C
CIV	Complex IV
CRYZ	Quinone oxidoreductase
CS	Citrate synthase
CT	Creatinine
DAP	Death-associated protein 1
DAPK1	Death-Associated Protein Kinase 1
DAPI	4’,6-diamidino-2-phenylindole
DCPIP	2,6-Dichlorophenolindophenol
DDA	Data-dependent acquisition
DTNB	5,5’-Dithiobis-(2-nitrobenzoic acid)
DUH2	Dihydrogen (reduced form)
DUB	Decylubiquinone
EDTA	Ethylenediaminetetraacetic acid
ELISA	Enzyme-linked immunosorbent assay
ER	Endoplasmic reticulum
ERAD	Endoplasmic reticulum-associated degradation
FBS	Fetal bovine serum
FCCP	Carbonyl cyanide-4-(trifluoromethoxy)phenylhydrazone
FCDGC	Frontier in CDG Consortium
FOXO3	Forkhead box protein O3
GC/M;S	Gas chromatography/mass spectrometry
HAPLN1	Hyaluronan and proteoglycan link protein 1
HCD	Higher-energy collisional dissociation
HCD	method: Higher-energy collisional dissociation method
IRB	Institutional Review Board
K2HPO4	Potassium hydrogen phosphate
K3Fe(CN)6	Potassium ferricyanide
LC-MS/MS	Liquid chromatography-tandem mass spectrometry
LC3	Light chain 3
LAMP1	Lysosome-associated membrane glycoprotein 1
LAMP2	Lysosome-associated membrane glycoprotein 2
LLO	Lipid-linked oligosaccharide
LRPPRC	Leucine-rich PPR motif-containing protein
Man-1-P	Mannose-1-phosphate
MDH1	Malate dehydrogenase, cytoplasmic
MESO	QuickPlex SQ 120: A specific instrument for measuring biomarkers
Mesoscale Discovery	A company specializing in measurement solutions
MPI	Phosphomannose isomerase
MRM3	rRNA methyltransferase 3
mtETC	Mitochondrial electron transport chain
NADH	Nicotinamide adenine dinucleotide
NaAz	Sodium azide
NIPSNAP3A	Protein NipSnap homolog 3A
NPCRS	Nijmegen Progression CDG Rating Scale
PINK1/PRKN	PTEN-induced putative kinase 1/parkin
PGM1-CDG	Phosphoglucomutase 1 Congenital Disorders of Glycosylation
PIGP-CDG	Phosphatidylinositol glycan anchor biosynthesis class P Congenital Disorders of Glycosylation
PKD2	Polycystin-2
PRDX6	Peroxiredoxin-6
QH2	Ubiquinone reduced to ubiquinol
RIPA	Radioimmunoprecipitation assay
SEC	Size-exclusion chromatography
SH3GLB1	Endophilin-B1
SLC39A8-CDG	Solute carrier family 39 member 8 Congenital Disorders of Glycosylation
SPATA18	Mitochondria-eating protein
sulfo-tag	Sulfo-tagged secondary antibody
TCA	Tricarboxylic acid
TBST	Tris-buffered saline with Tween 20
TEAB	Triethylammonium bicarbonate
Thermo Fisher Scientific	A scientific research company
TMT	Tandem mass tag
TNB	2-Nitro-5-thiobenzoate (anion)
TNB2	2-Nitro-5-thiobenzoate (dianion)
TPCK	Tosylphenylalanyl chloromethyl ketone
Tris-HCl	Tris(hydroxymethyl)aminomethane hydrochloride
TUBA1C	Tubulin α-1C chain
UBE2L3	Ubiquitin-conjugating enzyme E2 L3
UBE2N	Ubiquitin-conjugating enzyme E2 N
UFD1	Ubiquitin recognition factor in ER-associated degradation protein 1
UPR	Unfolded protein response
UBXN6	UBX domain-containing protein 6
VPS25	Vacuolar protein-sorting-associated protein 25
VPS4B	Vacuolar protein-sorting-associated protein 4B