| Symbol |
Name |
|
AARS2
|
alanyl-tRNA synthetase 2, mitochondrial
|
|
ACAD9
|
acyl-CoA dehydrogenase family, member 9
|
|
ADCK3
|
aarF domain containing kinase 3
|
|
AGK
|
acylglycerol kinase
|
|
ALDOB
|
aldolase B, fructose-bisphosphate
|
|
ATP5E
|
ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit
|
|
ATPAF2
|
ATP synthase mitochondrial F1 complex assembly factor 2
|
|
BCKDHA
|
branched chain keto acid dehydrogenase E1, alpha polypeptide
|
|
BCKDHB
|
branched chain keto acid dehydrogenase E1, beta polypeptide
|
|
BCS1L
|
BC1 (ubiquinol-cytochrome c reductase) synthesis-like
|
|
BOLA3
|
bolA family member 3
|
|
CA5A
|
carbonic anhydrase VA, mitochondrial
|
|
COA5
|
cytochrome c oxidase assembly factor 5
|
|
COQ2
|
coenzyme Q2 4-hydroxybenzoate polyprenyltransferase
|
|
COQ9
|
coenzyme Q9
|
|
COX10
|
COX10 heme A:farnesyltransferase cytochrome c oxidase assembly factor
|
|
COX14
|
COX14 cytochrome c oxidase assembly factor
|
|
COX15
|
cytochrome c oxidase assembly homolog 15 (yeast)
|
|
COX20
|
COX20 cytochrome c oxidase assembly factor
|
|
COX6B1
|
cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous)
|
|
CYC1
|
cytochrome c-1
|
|
DBT
|
dihydrolipoamide branched chain transacylase E2
|
|
DGUOK
|
deoxyguanosine kinase
|
|
DLAT
|
dihydrolipoamide S-acetyltransferase
|
|
DLD
|
dihydrolipoamide dehydrogenase
|
|
DNM1L
|
dynamin 1-like
|
|
EARS2
|
glutamyl-tRNA synthetase 2, mitochondrial
|
|
ELAC2
|
elaC ribonuclease Z 2
|
|
ETHE1
|
ethylmalonic encephalopathy 1
|
|
FARS2
|
phenylalanyl-tRNA synthetase 2, mitochondrial
|
|
FASTKD2
|
FAST kinase domains 2
|
|
FBXL4
|
F-box and leucine-rich repeat protein 4
|
|
FH
|
fumarate hydratase
|
|
FOXRED1
|
FAD-dependent oxidoreductase domain containing 1
|
|
G6PC
|
glucose-6-phosphatase, catalytic subunit
|
|
HADHA
|
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
|
|
HADHB
|
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
|
|
HSD17B10
|
hydroxysteroid (17-beta) dehydrogenase 10
|
|
IBA57
|
IBA57, iron-sulfur cluster assembly homolog (S. cerevisiae)
|
|
ISCU
|
iron-sulfur cluster assembly enzyme
|
|
LIAS
|
lipoic acid synthetase
|
|
LRPPRC
|
leucine-rich pentatricopeptide repeat containing
|
|
LYRM4
|
LYR motif containing 4
|
|
LYRM7
|
LYR motif containing 7
|
|
MLYCD
|
malonyl-CoA decarboxylase
|
|
MPC1
|
mitochondrial pyruvate carrier 1
|
|
MPV17
|
MpV17 mitochondrial inner membrane protein
|
|
MRPS16
|
mitochondrial ribosomal protein S16
|
|
MTO1
|
mitochondrial tRNA translation optimization 1
|
|
NDUFA1
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa
|
|
NDUFA10
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa
|
|
NDUFA11
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa
|
|
NDUFA12
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12
|
|
NDUFA2
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa
|
|
NDUFA9
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
|
|
NDUFAF1
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 1
|
|
NDUFAF2
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 2
|
|
NDUFAF3
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 3
|
|
NDUFAF4
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 4
|
|
NDUFAF5
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 5
|
|
NDUFAF6
|
NADH dehydrogenase (ubiquinone) complex I, assembly factor 6
|
|
NDUFB3
|
NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa
|
|
NDUFB9
|
NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa
|
|
NDUFS1
|
NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
|
|
NDUFS2
|
NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)
|
|
NDUFS3
|
NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)
|
|
NDUFS4
|
NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
|
|
NDUFS6
|
NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase)
|
|
NDUFS7
|
NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)
|
|
NDUFS8
|
NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
|
|
NDUFV1
|
NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
|
|
NDUFV2
|
NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa
|
|
NFU1
|
NFU1 iron-sulfur cluster scaffold
|
|
NUBPL
|
nucleotide binding protein-like
|
|
OGDH
|
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
|
|
PC
|
pyruvate carboxylase
|
|
PCCA
|
propionyl CoA carboxylase, alpha polypeptide
|
|
PCCB
|
propionyl CoA carboxylase, beta polypeptide
|
|
PDHA1
|
pyruvate dehydrogenase (lipoamide) alpha 1
|
|
PDHB
|
pyruvate dehydrogenase (lipoamide) beta
|
|
PDHX
|
pyruvate dehydrogenase complex, component X
|
|
PDP1
|
pyruvate dehyrogenase phosphatase catalytic subunit 1
|
|
PET100
|
PET100 homolog (S. cerevisiae)
|
|
PHKG2
|
phosphorylase kinase, gamma 2 (testis)
|
|
PUS1
|
pseudouridylate synthase 1
|
|
RMND1
|
required for meiotic nuclear division 1 homolog (S. cerevisiae)
|
|
RRM2B
|
ribonucleotide reductase M2 B (TP53 inducible)
|
|
SCO2
|
SCO2 cytochrome c oxidase assembly protein
|
|
SDHA
|
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
|
|
SDHAF1
|
succinate dehydrogenase complex assembly factor 1
|
|
SERAC1
|
serine active site containing 1
|
|
SFXN4
|
sideroflexin 4
|
|
SLC25A19
|
solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19
|
|
SLC25A3
|
solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3
|
|
SLC25A4
|
solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4
|
|
SLC37A4
|
solute carrier family 37 (glucose-6-phosphate transporter), member 4
|
|
SUCLA2
|
succinate-CoA ligase, ADP-forming, beta subunit
|
|
SUCLG1
|
succinate-CoA ligase, alpha subunit
|
|
SURF1
|
surfeit 1
|
|
TACO1
|
translational activator of mitochondrially encoded cytochrome c oxidase I
|
|
TAZ
|
tafazzin
|
|
TK2
|
thymidine kinase 2, mitochondrial
|
|
TMEM70
|
transmembrane protein 70
|
|
TPK1
|
thiamin pyrophosphokinase 1
|
|
TRMU
|
tRNA 5-methylaminomethyl-2-thiouridylate methyltransferase
|
|
TSFM
|
Ts translation elongation factor, mitochondrial
|
|
TUFM
|
Tu translation elongation factor, mitochondrial
|
|
TYMP
|
thymidine phosphorylase
|
|
VTI1A
|
vesicle transport through interaction with t-SNAREs 1A
|
|
YARS2
|
tyrosyl-tRNA synthetase 2, mitochondrial
|
