The ketogenic diet as a therapeutic intervention strategy in mitochondrial disease
Under a Creative Commons license
open access
Abbreviations
ACA
acetoacetate
AcCoA
acetyl coenzyme A
AMPK
AMP-activated protein kinase
β-HAD
β-hydroxyacyl-CoA dehydrogenase
BHB
β-hydroxybutyrate
C10
decanoic acid
CI-CV
complex I to complex V
CS
citrate synthase
CAT
catalase
ETC
electron transport chain
ETF
electron-transferring flavoprotein
FA
fatty acid
FADH2
flavin adenine dinucleotide
FAO
fatty acid oxidation
FATP
fatty acid transfer protein
FGF21
Fibroblast growth factor 21
GCL
glutamate cysteine ligase
GLUTs
glucose transporters
GPX
glutathione peroxidase
GR
glutathione reductase
GSH
reduced glutathione
GS
glutathione synthase
GSSG
oxidized glutathione
HDACs
histone deacetylases
KD
ketogenic diet
LCAD
long-chain acyl-CoA dehydrogenase
LS
Leigh syndrome
LHON
Leber’s hereditary optic neuropathy
LKB1
liver kinase B1
mAD
modified Atkins Diet
MCAD
medium-chain acyl-CoA dehydrogenase
MCT-KD
medium-chain triglyceride ketogenic diet
Mfn2
mitofusin 2
MD
mitochondrial disease
MELAS
Mitochondrial encephalopathy lactic acidosis, and stroke-like episodes
MIM
mitochondrial inner membrane
mPT
mitochondrial permeability transition
mPTP
mitochondrial permeability transition pore
mTORC1
mammalian target of rapamycin complex 1
mtDNA
mitochondrial DNA
NAD+
oxidized nicotinamide adenine dinucleotide
NADH
reduced nicotinamide adenine dinucleotide
NAMPT
nicotinamide phosphoribosyltransferase
nDNA
nuclear DNA
NNT
nicotinamide nucleotide transhydrogenase
NRF2
nuclear factor erythroid-derived 2-related factor 2
OXPHOS
oxidative phosphorylation
PARP
polyADP ribose polymerase
PEO
Progressive external ophthalmoplegia
PI3K
Phosphatidylinositol 3-kinase
PPP
pentose phosphate pathway
PRDX3
peroxiredoxin 3
ROS
reactive oxygen species
SCFAs
short chain fatty acids
SOD
superoxide dismutase
TCA
tricarboxylic acid
TRXR2
thioredoxin reductase 2
TRXS2
oxidized thioredoxin
TRX(SH)2
reduced thioredoxin
UCPs
uncoupling proteins
VLCAD
very-long-chain acyl-CoA dehydrogenase
Keywords
Mitochondria
Mitochondrial disease
Ketone bodies
Bioenergetics
Cited by (0)
© 2021 The Author(s). Published by Elsevier Ltd.