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Functions
Targets

Calcium-dependent
Delay in initiation of deloparization
Hyperpolarization

Opening of permeability transition pore (PTP)

Nonprotonophoric Uncouplers
Protonophoric uncoupling
Electrophoretic uncoupling

Transport of protons and charge (proton ionophore)
Transport of charge but not protons
Transport of protons but not charge (K+)
Transport of protons but not charge (Na+)
Transport of protons but not charge (Ca2+ or Mg2+)
Transport of protons but not charge (Cations)

Associate with uncoupling
Membrane potential
Redox cycling

proton conductivity by F0

State 1 respiration
State 2 respiration
State 3 respiration
State 4 respiration
State 5 respiration
State 3u respiration

Respiratory control ratio (RCR)
Oxygen consumption rate (OCR)
Basal respiration
Maximal respiration
ATP turnover
Spare/ respiratory capacity
Proton leak
Non-mitochondrial respiration
P/O ratio ( ADP/O)
Electron transport chain
Uncoupled respiration
DNP-uncoupled respiration

Generation of lactate
Lactate to pyruvate ratio

Generation of citrate
Generation of isocitrate
Generation of alpha-ketoglutarate
Generation of succinate

ECAR
Glucose consumption
Lactate production
Gluconeogenesis
Glucose galactose IC50 ratio
Pentose phosphate pathway

Mitochondrial fatty acid beta oxidation
Fatty acyl-CoA biosynthesis
Carnitine shuttle
Carnitine biosynthesis
Fatty acid biosynthetic
Acetyl-CoA biosynthesis

Synthesis of ketone body
Catabolism of ketone body
Lipid peroxidation
Steroid metabolism
Phospholipid metabolism

Alanine metabolism
Arginine metabolism
Asparagine metabolism
Aspartate metabolism
Cysteine metabolism
Glutamine metabolism
Glutamate metabolism
Glycine metabolism
Histidine metabolism
Isoleucine metabolism
Leucine metabolism
Lysine metabolism
Methionine metabolism
Phenylalanine metabolism
Proline metabolism
Serine metabolism
Threonine metabolism
Tryptophan metabolism
Tyrosine metabolism
Valine metabolism
Selenocysteine metabolism
Glycogen metabolism
Protein scaffolding

Mitochondrial ubiquitination

Malate asparatate shuttle
S,n-glycerophosphate shuttle
Transport of glutamate
Transport of dicarboxylate
Transport of 2-oxoglutarate
Carnitine shuttle
Transport of tricarboxylate
Transport of ornithine/citrulline
Transport of deoxynucleotide
Transport of L-aspartic acid
Transport of pyruvate
Transport of monocarboxylate
Transport of citrate
Transport of glucose
Transport of fatty acid
Mitochondrial protein transport

Transport of calcium
Accumulation of calcium
Transport of sodium
Transport of potassium
Potassium release
Accumulation of potassium
Transport of proton
Transport of phosphate
Accumulation of phosphate
Transport of chlorine
Transport of anion
Bicarbonate transport

ATP synthesis
NADPH metabolism
ATP level
ATP hydrolysis
Heme synthesis
CoA synthesis
Ubiquinone synthesis
Cobalamin (Cbl, vitamin B12) metabolism
Folate (vitamin B9) metabolism
Calciferol (Vitamin D) metabolism
Biotin (vitamin B7) metabolism
Thiamine (vitamin B1) metabolism
Riboflavin (vitamin B2) metabolism
NADH level
Energy charge
ATP/ADP ratio
Thiol compounds metabolism

Ferritin depletion

Phosphatidylethanolamine biosynthesis
Glyoxylate metabolim
Creatine metabolism
Lipoic acid metabolism

Pyrimidine nucleotide biosynthesis
Pyridine nucleotide biosynthesis

GSH content
GSSH/GHS ratio
Sulfur compound metabolism
Urea cycle

Fructose biosynthesis

Mass
Size
Swelling
Localization

Elongation

Volume
Formation and maintenance of cristae structure
Fragmentation
Megamitochondria

Tubulization
Structure integrity

Mitophagy
Biogenesis
Fusion
Fission
ER-mitochondria tethering

Mitochondrial respiratory chain complex assembly

Iron-sulfur cluster assembly

Anterograde axonal transport of mitochondrion
Retrograde axonal transport of mitochondrion
Mitochondrial motility

Heart-specific oxidative stress
Liver-specific oxidative stress
Neuron-specific oxidative stress

H2O2 production

Antioxidant production
Scavenger of mitochondrial superoxide
RSS production

Mitochondrial DNA metabolic process
Mitochondrial DNA metabolic process
Mitochondrial DNA metabolic process
Mitochondrial DNA metabolic process
Mitochondrial DNA metabolic process
Mitochondrial DNA mutation

Mitochondrial transcription
Mitochondrial mRNA processing
Mitochondrial tRNA processing
Mitochondrial rRNA processing

Mitochondrial translational elongation
Mitochondrial DNA damage

Early apoptosis
Late apoptosis
P53 pathway
Caspase activity
Cytochrome c release
ER stress-induced
Ferroptosis
Autophagy
Necroptosis
Pyroptosis

MTOR signaling pathway
AMPK signaling pathway
HIF-1 signaling pathway
MAPK signaling pathway
FoxO signaling pathway
JAK-STAT signaling pathway
ErbB signaling pathway
PI3K-Akt signaling pathway
Wnt signaling pathway
VEGF signaling pathway
Ras signaling pathway

NADH-ubiquinone oxidoreductase chain 1
NADH-ubiquinone oxidoreductase chain 2
NADH-ubiquinone oxidoreductase chain 3
NADH-ubiquinone oxidoreductase chain 4
NADH-ubiquinone oxidoreductase chain 4L
NADH-ubiquinone oxidoreductase chain 5
NADH-ubiquinone oxidoreductase chain 6
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial
NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial
NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial
Complex I intermediate-associated protein 30, mitochondrial
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4
Arginine-hydroxylase NDUFAF5, mitochondrial
NADH dehydrogenase (ubiquinone) complex I, assembly factor 6
Protein arginine methyltransferase NDUFAF7, mitochondrial
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 8
Complex I assembly factor ACAD9, mitochondrial
Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial
FAD-dependent oxidoreductase domain-containing protein 1
Iron-sulfur protein NUBPL
Complex I assembly factor TIMMDC1, mitochondrial
Complex I assembly factor TMEM126B, mitochondrial
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1
NADH dehydrogenase [ubiquinone] iron-sulfur protein 5
NADH dehydrogenase [ubiquinone] 1 subunit C2
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4
NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial
NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial
Acyl carrier protein, mitochondrial
Quinone
Semiquinone
Quinol
Flavin mononucleotide
Iron-sulfur clusters

Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
Succinate dehydrogenase assembly factor 1, mitochondrial
Succinate dehydrogenase assembly factor 2, mitochondrial
Quinone pocket
Succinate pocket
Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
Coenzyme Q10

Cytochrome b
Cytochrome b-c1 complex subunit 2, mitochondrial
Cytochrome b5 outer mitochondrial membrane isoform
Cytochrome b-c1 complex subunit 7
Cytochrome c1, heme protein, mitochondrial
Mitochondrial chaperone BCS1
Mitogen-activated protein kinase kinase kinase 20
Tetratricopeptide repeat protein 19, mitochondrial
Complex III assembly factor LYRM7
Ubiquinol-cytochrome-c reductase complex assembly factor 2
Ubiquinol-cytochrome-c reductase complex assembly factor 3
Qo site (Qp site or ubiquinol oxidation site)
Qi site (Qn site or quinone reduction site)
Cytochrome b-c1 complex subunit Rieske, mitochondrial
Cytochrome b-c1 complex subunit 8
Cytochrome b-c1 complex subunit 10
Cytochrome b-c1 complex subunit 9
Cytochrome b-c1 complex subunit 6, mitochondrial
Cytochrome b-c1 complex subunit 1, mitochondrial

Cytochrome c oxidase subunit 1
Cytochrome c oxidase subunit 2
Cytochrome c oxidase subunit 3
Cytochrome c oxidase subunit 4 isoform 1, mitochondrial
Cytochrome c oxidase subunit 4 isoform 2, mitochondrial
Cytochrome c oxidase subunit 5A, mitochondrial
Cytochrome c oxidase subunit 6A1, mitochondrial
Cytochrome c oxidase subunit 6B1
Cytochrome c oxidase subunit 7B, mitochondrial
Cytochrome c oxidase subunit 8A, mitochondrial
Cytochrome c oxidase subunit NDUFA4
Cytochrome c oxidase assembly factor 5
Cytochrome c oxidase assembly factor 6 homolog
Cytochrome c oxidase assembly factor 7
Cytochrome c oxidase assembly protein COX14
Cytochrome c oxidase assembly protein COX20, mitochondrial
Cytochrome c oxidase subunit 5B, mitochondrial
Cytochrome c oxidase subunit 6A2, mitochondrial
Cytochrome c oxidase subunit 6C
Cytochrome c oxidase subunit 7A1, mitochondrial
Cytochrome c oxidase subunit 7C, mitochondrial
Surfeit locus protein 1
Protein SCO1 homolog, mitochondrial
Protein SCO2 homolog, mitochondrial
Protoheme IX farnesyltransferase, mitochondrial
Cytochrome c oxidase assembly protein COX15 homolog
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
FAST kinase domain-containing protein 2, mitochondrial
Protein PET100 homolog, mitochondrial
Protein PET117 homolog, mitochondrial
Centrosomal protein of 89 kDa
Oxygen
Copper

C ring

ATP synthase F(0) complex subunit C1, mitochondrial

ATP synthase subunit gamma, mitochondrial
ATP synthase subunit delta, mitochondrial
ATP synthase subunit alpha, mitochondrial
ATP synthase subunit beta, mitochondrial
ATP synthase subunit O, mitochondrial
ATP synthase subunit g, mitochondrial
ATP synthase subunit e, mitochondrial
ATP synthase subunit epsilon, mitochondrial
ATP synthase mitochondrial F1 complex assembly factor 2
Transmembrane protein 70, mitochondrial
ATP synthase membrane subunit DAPIT, mitochondrial
ATP synthase subunit a
ATP synthase protein 8
V-type proton ATPase catalytic subunit A
ATP

Carnitine O-palmitoyltransferase 1, liver isoform, CPT1-L, EC 2.3.1.21
Carnitine O-palmitoyltransferase 1, muscle isoform
Carnitine O-palmitoyltransferase 1, brain isoform, CPT1-B, EC 2.3.1.21
Carnitine O-palmitoyltransferase 2, mitochondrial, EC 2.3.1.21
Mitochondrial carnitine/acylcarnitine carrier protein
Enoyl-CoA hydratase, mitochondrial, EC 4.2.1.17
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial, HCDH, EC 1.1.1.35
3-ketoacyl-CoA thiolase, mitochondrial

Long-chain-fatty-acid--CoA ligase 1
Long-chain-fatty-acid--CoA ligase 3
Long-chain-fatty-acid--CoA ligase 4
Long-chain-fatty-acid--CoA ligase 5
Long-chain-fatty-acid--CoA ligase 6
Long-chain-fatty-acid--CoA ligase ACSBG1
Long-chain-fatty-acid--CoA ligase ACSBG2
Very long-chain acyl-CoA synthetase
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Acetate CoA-transferase
Medium-chain acyl-CoA ligase ACSF2, mitochondrial
Carnitine O-acetyltransferase

Electron transfer flavoprotein subunit alpha, mitochondrial
Electron transfer flavoprotein beta subunit lysine methyltransferase
Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
Fatty acid 2-hydroxylase
3-hydroxyacyl-CoA dehydrogenase type-2
Pyrroline-5-carboxylate reductase 1, mitochondrial
Tricarboxylate transport protein, mitochondrial
Trifunctional enzyme subunit alpha, mitochondrial
Cytochrome P450 11B2, mitochondrial
Phosphatidylserine decarboxylase proenzyme, mitochondrial
Estradiol 17-beta-dehydrogenase 8
Hydroxymethylglutaryl-CoA lyase, mitochondrial
Acetyl-CoA carboxylase 2
Cholesterol side-chain cleavage enzyme, mitochondrial
Enoyl-CoA delta isomerase 2
Cytochrome P450 11B1, mitochondrial
Malonyl-CoA-acyl carrier protein transacylase, mitochondrial
Enoyl-CoA delta isomerase 1, mitochondrial
Glutaryl-CoA dehydrogenase, mitochondrial
2,4-dienoyl-CoA reductase, mitochondrial
D-beta-hydroxybutyrate dehydrogenase, mitochondrial
Isovaleryl-CoA dehydrogenase, mitochondrial
Acetyl-CoA acetyltransferase, mitochondrial
Succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial
Trimethyllysine dioxygenase, mitochondrial
Adrenodoxin, mitochondrial
Cytochrome P450 1A1
Cytochrome P450 2E1
Cytochrome P450 2C8
Cytochrome P450 2D6
Cytochrome P450 1B1
Solute carrier family 2, facilitated glucose transporter member 1
Hexokinase-1
Glucose-6-phosphate isomerase

ATP-dependent 6-phosphofructokinase, liver type
ATP-dependent 6-phosphofructokinase, muscle type
ATP-dependent 6-phosphofructokinase, platelet type

Fructose-bisphosphate aldolase A
Fructose-bisphosphate aldolase B
Triosephosphate isomerase
Glyceraldehyde-3-phosphate dehydrogenase
Phosphoglycerate kinase
Phosphoglycerate mutase
Enolase

L-lactate dehydrogenase A chain (LDH-A) (EC 1.1.1.27) (Cell proliferation-inducing gene 19 protein) (LDH muscle subunit) (LDH-M) (Renal carcinoma antigen NY-REN-59)
L-lactate dehydrogenase A-like 6B (EC 1.1.1.27)
L-lactate dehydrogenase B chain (LDH-B) (EC 1.1.1.27) (LDH heart subunit) (LDH-H) (Renal carcinoma antigen NY-REN-46)
L-lactate dehydrogenase C chain (LDH-C) (EC 1.1.1.27) (Cancer/testis antigen 32) (CT32) (LDH testis subunit) (LDH-X)
Lactate
Acetyl-coenzyme A synthetase 2-like, mitochondrial
Sorbitol dehydrogenase

[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial (EC 2.7.11.2) (Pyruvate dehydrogenase kinase isoform 1) (PDH kinase 1)
[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 2, mitochondrial (EC 2.7.11.2) (Pyruvate dehydrogenase kinase isoform 2) (PDH kinase 2) (PDKII)
[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial (EC 2.7.11.2) (Pyruvate dehydrogenase kinase isoform 3)
[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 4, mitochondrial (EC 2.7.11.2) (Pyruvate dehydrogenase kinase isoform 4)
Pyruvate kinase PKLR
Dihydrolipoyl dehydrogenase, mitochondrial (EC 1.8.1.4) (Dihydrolipoamide dehydrogenase) (Glycine cleavage system L protein)
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
Mitochondrial pyruvate carrier 1
Pyruvate carboxylase, mitochondrial

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
Pyruvate dehydrogenase protein X component, mitochondrial
[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial
Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrial
Hydroxyacylglutathione hydrolase, mitochondrial
Citrate synthase, mitochondrial
Aconitate hydratase, mitochondrial
Isocitrate dehydrogenase [NADP] cytoplasmic

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
2-oxoglutarate dehydrogenase, mitochondrial

Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial
Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial
Fumarate hydratase, mitochondrial
Malate dehydrogenase, cytoplasmic
Malate dehydrogenase, mitochondrial
Malate:quinone oxidoreductase
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
NAD(P)H-hydrate epimerase
NADP-dependent malic enzyme, mitochondrial
NAD-dependent malic enzyme, mitochondrial
Citrate
Isocitrate
Alpha-ketoglutarate
Succinate
Aspartate aminotransferase, mitochondrial
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Alanine--glyoxylate aminotransferase 2, mitochondrial
Glycine cleavage system H protein, mitochondrial
Transketolase
Phosphoenolpyruvate carboxykinase [GTP], mitochondrial

Creatine kinase U-type, mitochondrial
Creatine kinase S-type, mitochondrial
Glycogen synthase kinase-3 beta
Peptidyl-prolyl cis-trans isomerase F, mitochondrial
60 kDa heat shock protein, mitochondrial
Stress-70 protein, mitochondrial
Heat shock protein HSP 90-alpha
Heat shock protein HSP 90-beta
Heat shock protein 75 kDa, mitochondrial
Bifunctional coenzyme A synthase
Pantothenate kinase 2, mitochondrial
Mitochondrial coenzyme A transporter SLC25A42
4-hydroxybenzoate polyprenyltransferase, mitochondrial
Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial
2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial
Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial
5-demethoxyubiquinone hydroxylase, mitochondrial
Atypical kinase COQ8A, mitochondrial
Atypical kinase COQ8B, mitochondrial
Ubiquinone biosynthesis protein COQ9, mitochondrial

Decaprenyl-diphosphate synthase subunit 1
Decaprenyl-diphosphate synthase subunit 2
Corrinoid adenosyltransferase
Methylmalonyl-CoA mutase, mitochondrial
Folylpolyglutamate synthase, mitochondrial
Methylmalonic aciduria type A protein, mitochondrial
25-hydroxyvitamin D-1 alpha hydroxylase, mitochondrial
Dihydrofolate reductase 2, mitochondrial
Folic acid synthesis protein FOL1 [Includes: Dihydroneopterin aldolase
Bifunctional methylenetetrahydrofolate dehydrogenase/cyclohydrolase, mitochondrial
NADPH:adrenodoxin oxidoreductase, mitochondrial
Ferredoxin-2, mitochondrial
Frataxin, mitochondrial
Iron-sulfur cluster assembly 1 homolog, mitochondrial
Iron-sulfur cluster assembly 2 homolog, mitochondrial
Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
LYR motif-containing protein 4
Cysteine desulfurase, mitochondrial
NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
BolA-like protein 3
Glutaredoxin-related protein 5, mitochondrial
Putative transferase CAF17, mitochondrial
Lipoyl synthase, mitochondrial
Lipoyltransferase 1, mitochondrial
Putative lipoyltransferase 2, mitochondrial
Enoyl-[acyl-carrier-protein] reductase, mitochondrial
Biotinidase
Biotin--protein ligase

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Propionyl-CoA carboxylase beta chain, mitochondrial
Propionyl-CoA carboxylase alpha chain, mitochondrial
Thiamine transporter 1
Thiamine transporter 2
Thiamin pyrophosphokinase 1
S-adenosylmethionine mitochondrial carrier protein
Mitochondrial folate transporter/carrier
Calcium-binding mitochondrial carrier protein SCaMC-1
Acetyl-coenzyme A transporter 1
Metal cation symporter ZIP8
Selenocysteine insertion sequence-binding protein 2
O-phosphoseryl-tRNA(Sec) selenium transferase
NAD kinase 2, mitochondrial
ATP-dependent (S)-NAD(P)H-hydrate dehydratase
NAD(P)H-hydrate epimerase
FAD synthase
Solute carrier family 52, riboflavin transporter, member 2
Solute carrier family 52, riboflavin transporter, member 3
MAS
Glycerol-3-phosphate dehydrogenase, mitochondrial
Mitochondrial glutamate carrier 2
Mitochondrial glutamate carrier 1
Mitochondrial dicarboxylate carrier
Mitochondrial 2-oxoglutarate/malate carrier protein
Calcium-binding mitochondrial carrier protein Aralar1
Calcium-binding mitochondrial carrier protein Aralar2
Mitochondrial basic amino acids transporter
Mitochondrial ornithine transporter 1
Mitochondrial ornithine transporter 2
Mitochondrial thiamine pyrophosphate carrier
ADP/ATP translocase 1
ADP/ATP translocase 2
ADP/ATP translocase 3
Mitochondrial pyruvate carrier
Monocarboxylate transporter 1
ATP-binding cassette sub-family B member 6, mitochondrial
ATP-binding cassette sub-family B member 7, mitochondrial
ATP-binding cassette sub-family B member 10, mitochondrial
ADP-ribosylation factor-like protein 2
Translocator protein
Glutaredoxin-2, mitochondrial

Mitochondrial import inner membrane translocase subunit Tim22
Mitochondrial import inner membrane translocase subunit Tim23

Mitochondrial import receptor subunit TOM22 homolog
Mitochondrial import receptor subunit TOM40 homolog
Mitochondrial import receptor subunit TOM20 homolog
MCU (mitochondrial calcium uniporter)
Mitochondrial Na/Ca2+ exchanger
Mitochondrial Na/H+ exchanger
Mitochondrial KATP chanel (mtKATP)
K+/H+ exchanger (KHE)
Potassium voltage-gated channel subfamily A member 3
Potassium voltage-gated channel subfamily A member 5

Potassium voltage-gated channel subfamily H member 2
Potassium voltage-gated channel subfamily H member 6
Potassium voltage-gated channel subfamily H member 7
Potassium
Phosphate carrier protein, mitochondrial
Chloride intracellular channel protein 4
Voltage-dependent anion-selective channel protein 1
Voltage-dependent anion-selective channel protein 2
Voltage-dependent anion-selective channel protein 3
Hexokinase-2
Mitochondrial uncoupling protein
Mitochondrial brown fat uncoupling protein 1
Mitochondrial uncoupling protein 2
Mitochondrial uncoupling protein 3
Mitochondrial uncoupling protein 4
Adenylate cyclase type 10
Peroxisomal acyl-coenzyme A oxidase 1
Thiol compounds
F-actin cytoskeleton
Tripeptidyl-peptidase 1
Aurora kinase B
DNA-directed RNA polymerase, mitochondrial
Mitochondrial topoisomerases
Transcription factor A, mitochondrial

DNA polymerase subunit gamma-1
DNA polymerase subunit gamma-2, mitochondrial
Mitochondrial thymidine kinase 2
Deoxyguanosine kinase
Twinkle mtDNA helicase
Single-stranded DNA-binding protein, mitochondrial
DNA ligase 3
Aprataxin
DNA repair protein XRCC4
DNA topoisomerase I, mitochondrial
Uracil-DNA glycosylase
4-aminobutyrate aminotransferase, mitochondrial
Adenylate kinase 2, mitochondrial
Ribonucleoside-diphosphate reductase subunit M2 B
Deoxynucleoside triphosphate triphosphohydrolase SAMHD1
Thymidine phosphorylase
5'(3')-deoxyribonucleotidase, mitochondrial
Dihydroorotate dehydrogenase
Dihydroorotate dehydrogenase
Nicotinamide/nicotinic acid mononucleotide adenylyltransferase 3
ADP-ribose pyrophosphatase, mitochondrial
DNA replication ATP-dependent helicase/nuclease DNA2
F-box/LRR-repeat protein 4
Mitochondrial genome maintenance exonuclease 1
Protein Mpv17
DNA polymerase subunit gamma-2, mitochondrial
Single-stranded DNA-binding protein, mitochondrial
Prohibitin-2
Thiosulfate sulfurtransferase
Alanine--tRNA ligase, mitochondrial
Probable cysteine--tRNA ligase, mitochondrial
Aspartate--tRNA ligase, cytoplasmic
Aspartate--tRNA ligase, mitochondrial
Probable glutamate--tRNA ligase, mitochondrial
Phenylalanine--tRNA ligase, mitochondrial
Glycine--tRNA ligase
Histidine--tRNA ligase, mitochondrial
Isoleucine--tRNA ligase, cytoplasmic
Isoleucine--tRNA ligase, mitochondrial
Lysine--tRNA ligase
Leucine--tRNA ligase, cytoplasmic
Probable leucine--tRNA ligase, mitochondrial
Methionine--tRNA ligase, mitochondrial
Probable asparagine--tRNA ligase, mitochondrial
Probable proline--tRNA ligase, mitochondrial
Glutamine--tRNA ligase
Probable arginine--tRNA ligase, mitochondrial
Serine--tRNA ligase, mitochondrial
Threonine--tRNA ligase, mitochondrial
Valine--tRNA ligase, mitochondrial
Tryptophan--tRNA ligase, mitochondrial
Tyrosine--tRNA ligase, mitochondrial
Zinc phosphodiesterase ELAC protein 2
Methionyl-tRNA formyltransferase, mitochondrial
TRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial
2',5'-phosphodiesterase 12
Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial
TRNA dimethylallyltransferase
TRNA (guanine(37)-N1)-methyltransferase
TRNA methyltransferase 10 homolog C
CCA tRNA nucleotidyltransferase 1, mitochondrial
Mitochondrial-derived peptide MOTS-c
Humanin
Polyribonucleotide nucleotidyltransferase 1, mitochondrial
Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial
GTPase Era, mitochondrial
39S ribosomal protein L3, mitochondrial
39S ribosomal protein L12, mitochondrial
39S ribosomal protein L44, mitochondrial
28S ribosomal protein S2, mitochondrial
28S ribosomal protein S7, mitochondrial
28S ribosomal protein S16, mitochondrial
28S ribosomal protein S22, mitochondrial
28S ribosomal protein S23, mitochondrial
28S ribosomal protein S34, mitochondrial
RRNA methyltransferase 2, mitochondrial
Required for meiotic nuclear division protein 1 homolog
Probable peptide chain release factor C12orf65, mitochondrial
Elongation factor G, mitochondrial
Ribosome-releasing factor 2, mitochondrial
TRNA modification GTPase GTPBP3, mitochondrial
Translation factor GUF1, mitochondrial
Leucine-rich PPR motif-containing protein, mitochondrial
Protein MTO1 homolog, mitochondrial
Poly(A) RNA polymerase, mitochondrial
TRNA pseudouridine synthase A
Translational activator of cytochrome c oxidase 1
Mitochondrial tRNA-specific 2-thiouridylase 1
Elongation factor Ts, mitochondrial
Elongation factor Tu, mitochondrial
Peptide deformylase, mitochondrial
Acylglycerol kinase, mitochondrial
Choline/ethanolamine kinase
Mitochondrial import inner membrane translocase subunit TIM14
FAD-linked sulfhydryl oxidase ALR
Mitochondrial intermediate peptidase
Mitochondrial import inner membrane translocase subunit TIM16
85/88 kDa calcium-independent phospholipase A2
Mitochondrial-processing peptidase subunit alpha
Protein SERAC1
Tafazzin
Mitochondrial import inner membrane translocase subunit Tim8 A
Mitochondrial import inner membrane translocase subunit TIM50
Xaa-Pro aminopeptidase 3
Dynamin-1-like protein
Ganglioside-induced differentiation-associated protein 1
Mitochondrial fission factor
Mitofusin-2
Protein misato homolog 1
Dynamin-like 120 kDa protein, mitochondrial
Signal transducer and activator of transcription 2
Trafficking kinesin-binding protein 1
ATP-dependent zinc metalloprotease YME1L1
Solute carrier family 25 member 46
ER membrane protein complex subunit 1
AFG3-like protein 2
ATPase family AAA domain-containing protein 3A
Caseinolytic peptidase B protein homolog
ATP-dependent Clp protease proteolytic subunit, mitochondrial
ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial
10 kDa heat shock protein, mitochondrial
Lon protease homolog, mitochondrial
Presequence protease, mitochondrial
Sacsin
Paraplegin
Heat shock protein 75 kDa, mitochondrial
ATP-dependent Clp protease proteolytic subunit, mitochondrial
Mitochondrial peptide methionine sulfoxide reductase
Persulfide dioxygenase ETHE1, mitochondrial
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
NAD(P) transhydrogenase, mitochondrial
Thioredoxin, mitochondrial

Superoxide
Hydrogen peroxide
Catalase
Glutathione reductase, mitochondrial
Glutathione synthetase
Peroxiredoxin-5, mitochondrial
Xanthine dehydrogenase/oxidase [Includes: Xanthine dehydrogenase
Myeloperoxidase
NADPH oxidase
NADPH oxidase 1
NADPH oxidase 4
Superoxide dismutase [Mn], mitochondrial
Phospholipid hydroperoxide glutathione peroxidase
Thioredoxin reductase 2, mitochondrial
Oxidized glutathione
Glycine N-acyltransferase
Apoptosis regulator Bcl-2
Apoptosis regulator BAX
Bcl-2-like protein 2
Bcl-2-like protein 1
Bcl2-associated agonist of cell death
Cytochrome c
Caspase-3
Serine/threonine-protein kinase PAK 5
Apoptosis-inducing factor 1, mitochondrial
Cytochrome c oxidase assembly factor 8
Diablo homolog, mitochondrial
Serine protease HTRA2, mitochondrial
Peptidyl-tRNA hydrolase 2, mitochondrial
Serine/threonine-protein kinase mTOR
5'-AMP-activated protein kinase catalytic subunit alpha-2
Hypoxia-inducible factor 1-alpha
Peroxisome proliferator-activated receptor gamma coactivator 1-alpha
Peroxisome proliferator-activated receptor gamma coactivator 1-beta
Cyclic AMP-responsive element-binding protein 1
NAD-dependent protein deacetylase sirtuin-1
NAD-dependent protein deacetylase sirtuin-3, mitochondrial
NAD-dependent protein lipoamidase sirtuin-4, mitochondrial
NAD-dependent protein deacylase sirtuin-5, mitochondrial
Mitogen-activated protein kinase 14
A-kinase anchor protein 1, mitochondrial
Anoctamin-10
MICOS complex subunit MIC13
CDGSH iron-sulfur domain-containing protein 2
1,25-dihydroxyvitamin D(3) 24-hydroxylase, mitochondrial
Calcium uptake protein 1, mitochondrial
Calcium uptake protein 2, mitochondrial
Wolframin
Arginase-2, mitochondrial
N-acetylglutamate synthase, mitochondrial
Carbamoyl-phosphate synthase [ammonia], mitochondrial
Glycine amidinotransferase, mitochondrial
Glutamate dehydrogenase 2, mitochondrial
Glycine N-acyltransferase-like protein 1
Proline dehydrogenase 1, mitochondrial
Serine hydroxymethyltransferase, mitochondrial
2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
Aminomethyltransferase, mitochondrial
Glycine dehydrogenase
Branched-chain-amino-acid aminotransferase, mitochondrial
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Isobutyryl-CoA dehydrogenase, mitochondrial
3-hydroxyisobutyrate dehydrogenase, mitochondrial
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
Alpha-aminoadipic semialdehyde synthase, mitochondrial
5-phosphohydroxy-L-lysine phospho-lyase
Glutamate dehydrogenase 1, mitochondrial
Ornithine aminotransferase, mitochondrial
Glutaminase liver isoform, mitochondrial
Ornithine carbamoyltransferase, mitochondrial
Succinate-semialdehyde dehydrogenase, mitochondrial
Amine oxidase [flavin-containing] B
Amine oxidase [flavin-containing] A
Aldehyde dehydrogenase, mitochondrial
5-aminolevulinate synthase, erythroid-specific, mitochondrial
Sideroflexin-4
Mitochondrial glycine transporter
5-aminolevulinate synthase, nonspecific, mitochondrial
5-aminolevulinate synthase
Ferrochelatase, mitochondrial
Aldehyde dehydrogenase X, mitochondrial
Delta-1-pyrroline-5-carboxylate synthase
D-beta-hydroxybutyrate dehydrogenase, mitochondrial
Carbonic anhydrase 5A, mitochondrial
C-terminal-binding protein 1
Complement component 1 Q subcomponent-binding protein, mitochondrial
Protein C19orf12
Netrin receptor DCC
Protein diaphanous homolog 1
Optic atrophy 3 protein
Patatin-like phospholipase domain-containing protein 4
Calcium-independent phospholipase A2-gamma
Inorganic pyrophosphatase 2, mitochondrial
Major prion protein
Sulfite oxidase, mitochondrial
Carbonic anhydrase 5B, mitochondrial
Endonuclease G, mitochondrial
Mitofusin-1
Mitochondrially Encoded TRNA-Lys (AAA/G)
Mitochondrially Encoded TRNA-Leu (UUA/G) 1
Mitochondrially Encoded TRNA-Ser (UCN) 1
Mitochondrially Encoded TRNA-Glu (GAA/G)
Mitochondrially Encoded 12S RRNA
Mitochondrially Encoded TRNA-Ile (AUU/C)
Mitochondrially Encoded TRNA-Leu (CUN) 2
Mitochondrially Encoded TRNA-Trp (UGA/G)
Mitochondrially Encoded TRNA-Phe (UUU/C)
Mitochondrially Encoded TRNA-Asn (AAU/C)
Mitochondrially Encoded TRNA-Val (GUN)
Mitochondrially Encoded TRNA-Ser (AGU/C) 2
Mitochondrially Encoded TRNA-Pro (CCN)
Mitochondrially Encoded TRNA-Thr (ACN)
Mitochondrially Encoded TRNA-His (CAU/C)
Mitochondrially Encoded TRNA-Gln (CAA/G)
Mitochondrially Encoded TRNA-Ala (GCN)
Mitochondrially Encoded TRNA-Cys (UGU/C)
Mitochondrially Encoded TRNA-Met (AUA/G)
Mitochondrially Encoded 16S RRNA
NDUFV2 Antisense RNA 1
Mitochondrially Encoded TRNA-Arg (CGN)
DGUOK Antisense RNA 1
Mitochondrially Encoded TRNA-Tyr (UAU/C)
COX10 Antisense RNA 1
Mitochondrially Encoded TRNA-Asp (GAU/C)
MicroRNA 9-1
OPA1 Antisense RNA 1
Mitochondrially Encoded TRNA-Gly (GGN)
TRNA-Leu (Anticodon AAG) 2-3
LARS2 Antisense RNA 1
ATXN8 Opposite Strand LncRNA
MicroRNA 132
RNA Component Of Mitochondrial RNA Processing Endoribonuclease
Small Nucleolar RNA, C/D Box 35A
MicroRNA 29a
MicroRNA 146a
WARS2 Antisense RNA 1
RNA, Ribosomal 45S Cluster 1
MicroRNA 23b
MicroRNA 133b
MicroRNA 328
MicroRNA 34a
RNA, U6atac Small Nuclear (U12-Dependent Splicing)
MicroRNA 210
MicroRNA 29b-1
MicroRNA 29c
MicroRNA 22
Small Nucleolar RNA, H/ACA Box 12
Small Nucleolar RNA, C/D Box 101
MicroRNA 17
Nuclear Paraspeckle Assembly Transcript 1
RNA, Ribosomal 45S Cluster 2
MicroRNA 126
MicroRNA 106b
MicroRNA 107
MicroRNA 125a
MicroRNA 433
MicroRNA 298
MicroRNA 323a
Small Nucleolar RNA, H/ACA Box 49
Vault RNA 2-1
LYRM4 Antisense RNA 1
MicroRNA 4432
MicroRNA 21
MicroRNA 483
MicroRNA 144
MicroRNA 124-1
Metastasis Associated Lung Adenocarcinoma Transcript 1
MicroRNA 196a-1
PINK1 Antisense RNA
MicroRNA 24-1
Small Nucleolar RNA, H/ACA Box 64
Uncharacterized LOC101928525
Long Intergenic Non-Protein Coding RNA 1734
MicroRNA 122
MicroRNA 26b
MicroRNA 151a
GABPB1 Intronic Transcript
Survival Associated Mitochondrial Melanoma Specific Oncogenic Non-Coding RNA
CDKN2B Antisense RNA 1
MicroRNA Let-7g
MicroRNA Let-7b
MicroRNA 142
MicroRNA 197
MicroRNA 92a-1
Small Nucleolar RNA Host Gene 3
MicroRNA 4721
Down Syndrome Critical Region 8
MicroRNA Let-7d
Highly Accelerated Region 1A
MicroRNA 212
Brain Cytoplasmic RNA 1
DNAJC9 Antisense RNA 1
Highly Accelerated Region 1B
MicroRNA 376a-1
Small Nucleolar RNA, C/D Box 97
Long Intergenic Non-Protein Coding RNA 841
Myosin Heavy Chain Associated RNA Transcript
Uncharacterized LOC102723385
Maternally Expressed 3
C1QTNF3-AMACR Readthrough (NMD Candidate)
MicroRNA 96
MicroRNA 494
MicroRNA 451a
HOX Transcript Antisense RNA
MicroRNA 128-1
MicroRNA 15a
MicroRNA 223
MicroRNA 320a
MicroRNA 155
BDNF Antisense RNA
H19 Imprinted Maternally Expressed Transcript
MicroRNA 20a
Long Intergenic Non-Protein Coding RNA 970
Small Nucleolar RNA Host Gene 1
MicroRNA 93
MicroRNA 324
Surfactant Associated 3
MicroRNA 519d
TCL1 Upstream Neural Differentiation-Associated RNA
MicroRNA 363
Telomerase RNA Component
GDF5 Antisense RNA 1
Myocardial Infarction Associated Transcript
MicroRNA 326
Prion Locus LncRNA, Testis Expressed
KCNQ1 Downstream Neighbor
MicroRNA 140
Prostate Cancer Associated 3
Taurine Up-Regulated 1
MicroRNA 150
SOX2 Overlapping Transcript
MIR155 Host Gene
MicroRNA Let-7i
DiGeorge Syndrome Critical Region Gene 5
MicroRNA 181c
RNA, U4atac Small Nuclear (U12-Dependent Splicing)
MicroRNA 183
MicroRNA 346
MicroRNA 330
MicroRNA 331
MicroRNA 296
MicroRNA 30d
MAPT Antisense RNA 1
MicroRNA 659
MicroRNA 103a-1
RNA, U1 Small Nuclear 1
MicroRNA 361
MicroRNA 299
HTT Antisense RNA
Uncharacterized LOC90024
MicroRNA 511
UCHL1 Antisense RNA 1 (Head To Head)
ZNF205 Antisense RNA 1
RNF217 Antisense RNA 1 (Head To Head)
Long Intergenic Non-Protein Coding RNA 1262
BACE1 Antisense RNA
SCOC Antisense RNA 1
Long Intergenic Non-Protein Coding RNA 1080
DNAH17 Antisense RNA 1
LRP1 Antisense RNA
FSIP2 Antisense RNA 1
MicroRNA 6084
Uncharacterized LOC100129175
MIR4422 Host Gene
Long Intergenic Non-Protein Coding RNA 1772
Long Intergenic Non-Protein Coding RNA 1616
MicroRNA 27a
TRNA-Pro (Anticodon AGG) 2-5
MicroRNA 195
MicroRNA 200a
MicroRNA 214
TIMM23B-AGAP6 Readthrough (NMD Candidate)
DAOA Antisense RNA 1
MicroRNA 7-1
MicroRNA 181a-1
MicroRNA 181b-1
MicroRNA 181b-2
SSBP3 Antisense RNA 1
Long Intergenic Non-Protein Coding RNA 1370
MicroRNA 548t
PTPRD Antisense RNA 2 (Head To Head)
Long Intergenic Non-Protein Coding RNA 1309
MicroRNA 7977
MicroRNA 30a
MicroRNA 29b-2
TRNA-Leu (Anticodon TAG) 1-1
MicroRNA 27b
MicroRNA 125b-1
MicroRNA 382
MicroRNA 590
Urothelial Cancer Associated 1
IGF2 Antisense RNA
MicroRNA 1304
MicroRNA 4738
MicroRNA 6878
MicroRNA 6890
MicroRNA 2909
MicroRNA 98
MicroRNA 663a
DISC1 Fusion Partner 1
Focally Amplified Long Non-Coding RNA In Epithelial Cancer
MicroRNA 593
MicroRNA 668
MicroRNA 199a-1
MicroRNA 101-1
MicroRNA 25
MicroRNA 15b
MicroRNA 199a-2
MicroRNA 148a
MicroRNA 31
MicroRNA 124-3
MicroRNA 138-1
MicroRNA 124-2
MicroRNA 570
MicroRNA 203a
MicroRNA 423
MicroRNA 634
RNA, 5.8S Ribosomal N1
RNA, 5.8S Ribosomal N2
RNA, 5.8S Ribosomal N3
SOD2 Overlapping Transcript 1
Uncharacterized LOC101929762
MicroRNA 143
MicroRNA 33a
MicroRNA 33b
MicroRNA 4485