| Symbol |
Name |
|
AADAT
|
aminoadipate aminotransferase
|
|
AASS
|
aminoadipate-semialdehyde synthase
|
|
ABAT
|
4-aminobutyrate aminotransferase
|
|
ACAD8
|
acyl-CoA dehydrogenase family, member 8
|
|
ACADL
|
acyl-CoA dehydrogenase, long chain
|
|
ACADM
|
acyl-CoA dehydrogenase, C-4 to C-12 straight chain
|
|
ACADS
|
acyl-CoA dehydrogenase, C-2 to C-3 short chain
|
|
ACADSB
|
acyl-CoA dehydrogenase, short/branched chain
|
|
ACADVL
|
acyl-CoA dehydrogenase, very long chain
|
|
ACAT1
|
acetyl-CoA acetyltransferase 1
|
|
ACO2
|
aconitase 2, mitochondrial
|
|
ACSM1
|
acyl-CoA synthetase medium-chain family member 1
|
|
ACSM2A
|
acyl-CoA synthetase medium-chain family member 2A
|
|
ACSM2B
|
acyl-CoA synthetase medium-chain family member 2B
|
|
ACSM3
|
acyl-CoA synthetase medium-chain family member 3
|
|
ACSM4
|
acyl-CoA synthetase medium-chain family member 4
|
|
ACSM5
|
acyl-CoA synthetase medium-chain family member 5
|
|
ACSS1
|
acyl-CoA synthetase short-chain family member 1
|
|
ADHFE1
|
alcohol dehydrogenase, iron containing, 1
|
|
ADPRHL2
|
ADP-ribosylhydrolase like 2
|
|
AGXT
|
alanine-glyoxylate aminotransferase
|
|
AGXT2
|
alanine--glyoxylate aminotransferase 2
|
|
AK3
|
adenylate kinase 3
|
|
ALAS1
|
5'-aminolevulinate synthase 1
|
|
ALAS2
|
5'-aminolevulinate synthase 2
|
|
ALDH1B1
|
aldehyde dehydrogenase 1 family, member B1
|
|
ALDH2
|
aldehyde dehydrogenase 2 family (mitochondrial)
|
|
ALDH4A1
|
aldehyde dehydrogenase 4 family, member A1
|
|
ALDH5A1
|
aldehyde dehydrogenase 5 family, member A1
|
|
ALDH6A1
|
aldehyde dehydrogenase 6 family, member A1
|
|
ALDH7A1
|
aldehyde dehydrogenase 7 family, member A1
|
|
ALKBH7
|
alkB, alkylation repair homolog 7 (E. coli)
|
|
ARG2
|
arginase 2
|
|
ATG4D
|
autophagy related 4D, cysteine peptidase
|
|
ATP5A1
|
ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle
|
|
ATP5B
|
ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide
|
|
ATP5C1
|
ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1
|
|
ATP5D
|
ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit
|
|
ATP5E
|
ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit
|
|
ATP5F1
|
ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1
|
|
ATXN3
|
ataxin 3
|
|
ATXN3L
|
ataxin 3-like
|
|
AUH
|
AU RNA binding protein/enoyl-CoA hydratase
|
|
BCAT2
|
branched chain amino-acid transaminase 2, mitochondrial
|
|
BCKDHA
|
branched chain keto acid dehydrogenase E1, alpha polypeptide
|
|
BCKDHB
|
branched chain keto acid dehydrogenase E1, beta polypeptide
|
|
BCL2L1
|
BCL2-like 1
|
|
BCO2
|
beta-carotene oxygenase 2
|
|
BDH1
|
3-hydroxybutyrate dehydrogenase, type 1
|
|
BLOC1S1
|
biogenesis of lysosomal organelles complex-1, subunit 1
|
|
BTD
|
biotinidase
|
|
C10ORF2
|
chromosome 10 open reading frame 2
|
|
C1QBP
|
complement component 1, q subcomponent binding protein
|
|
CA5A
|
carbonic anhydrase VA, mitochondrial
|
|
CA5B
|
carbonic anhydrase VB, mitochondrial
|
|
CARS2
|
cysteinyl-tRNA synthetase 2, mitochondrial (putative)
|
|
CASQ1
|
calsequestrin 1 (fast-twitch, skeletal muscle)
|
|
CBR4
|
carbonyl reductase 4
|
|
CCAR2
|
cell cycle and apoptosis regulator 2
|
|
CHPF
|
chondroitin polymerizing factor
|
|
CLPP
|
caseinolytic mitochondrial matrix peptidase proteolytic subunit
|
|
CLPX
|
caseinolytic mitochondrial matrix peptidase chaperone subunit
|
|
COASY
|
CoA synthase
|
|
COQ3
|
coenzyme Q3 methyltransferase
|
|
CPS1
|
carbamoyl-phosphate synthase 1, mitochondrial
|
|
CS
|
citrate synthase
|
|
CYP11A1
|
cytochrome P450, family 11, subfamily A, polypeptide 1
|
|
CYP27A1
|
cytochrome P450, family 27, subfamily A, polypeptide 1
|
|
D2HGDH
|
D-2-hydroxyglutarate dehydrogenase
|
|
DARS2
|
aspartyl-tRNA synthetase 2, mitochondrial
|
|
DBT
|
dihydrolipoamide branched chain transacylase E2
|
|
DECR1
|
2,4-dienoyl CoA reductase 1, mitochondrial
|
|
DGUOK
|
deoxyguanosine kinase
|
|
DHFRL1
|
dihydrofolate reductase-like 1
|
|
DHRS2
|
dehydrogenase/reductase (SDR family) member 2
|
|
DLAT
|
dihydrolipoamide S-acetyltransferase
|
|
DLD
|
dihydrolipoamide dehydrogenase
|
|
DLST
|
dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex)
|
|
DMGDH
|
dimethylglycine dehydrogenase
|
|
DNAJA3
|
DnaJ (Hsp40) homolog, subfamily A, member 3
|
|
DTYMK
|
deoxythymidylate kinase (thymidylate kinase)
|
|
DUSP21
|
dual specificity phosphatase 21
|
|
EARS2
|
glutamyl-tRNA synthetase 2, mitochondrial
|
|
ECHS1
|
enoyl CoA hydratase, short chain, 1, mitochondrial
|
|
ECI1
|
enoyl-CoA delta isomerase 1
|
|
ERAL1
|
Era-like 12S mitochondrial rRNA chaperone 1
|
|
ERBB4
|
erb-b2 receptor tyrosine kinase 4
|
|
ETFA
|
electron-transfer-flavoprotein, alpha polypeptide
|
|
ETFB
|
electron-transfer-flavoprotein, beta polypeptide
|
|
ETFDH
|
electron-transferring-flavoprotein dehydrogenase
|
|
ETHE1
|
ethylmalonic encephalopathy 1
|
|
FARS2
|
phenylalanyl-tRNA synthetase 2, mitochondrial
|
|
FDX1
|
ferredoxin 1
|
|
FDX1L
|
ferredoxin 1-like
|
|
FDXR
|
ferredoxin reductase
|
|
FECH
|
ferrochelatase
|
|
FH
|
fumarate hydratase
|
|
FLAD1
|
flavin adenine dinucleotide synthetase 1
|
|
FPGS
|
folylpolyglutamate synthase
|
|
FXN
|
frataxin
|
|
GADD45GIP1
|
growth arrest and DNA-damage-inducible, gamma interacting protein 1
|
|
GARS
|
glycyl-tRNA synthetase
|
|
GCDH
|
glutaryl-CoA dehydrogenase
|
|
GFM1
|
G elongation factor, mitochondrial 1
|
|
GFM2
|
G elongation factor, mitochondrial 2
|
|
GLS
|
glutaminase
|
|
GLS2
|
glutaminase 2 (liver, mitochondrial)
|
|
GLUD1
|
glutamate dehydrogenase 1
|
|
GLYAT
|
glycine-N-acyltransferase
|
|
GOT2
|
glutamic-oxaloacetic transaminase 2, mitochondrial
|
|
GPT2
|
glutamic pyruvate transaminase (alanine aminotransferase) 2
|
|
GPX1
|
glutathione peroxidase 1
|
|
GRPEL1
|
GrpE-like 1, mitochondrial (E. coli)
|
|
GRPEL2
|
GrpE-like 2, mitochondrial (E. coli)
|
|
GSR
|
glutathione reductase
|
|
GSTK1
|
glutathione S-transferase kappa 1
|
|
GUF1
|
GUF1 GTPase homolog (S. cerevisiae)
|
|
HADH
|
hydroxyacyl-CoA dehydrogenase
|
|
HAGH
|
hydroxyacylglutathione hydrolase
|
|
HARS2
|
histidyl-tRNA synthetase 2, mitochondrial
|
|
HIBADH
|
3-hydroxyisobutyrate dehydrogenase
|
|
HIBCH
|
3-hydroxyisobutyryl-CoA hydrolase
|
|
HMGCL
|
3-hydroxymethyl-3-methylglutaryl-CoA lyase
|
|
HMGCS2
|
3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)
|
|
HSD17B10
|
hydroxysteroid (17-beta) dehydrogenase 10
|
|
HSD17B8
|
hydroxysteroid (17-beta) dehydrogenase 8
|
|
HSPA1L
|
heat shock 70kDa protein 1-like
|
|
HSPD1
|
heat shock 60kDa protein 1 (chaperonin)
|
|
HSPE1
|
heat shock 10kDa protein 1
|
|
IARS2
|
isoleucyl-tRNA synthetase 2, mitochondrial
|
|
IBA57
|
IBA57, iron-sulfur cluster assembly homolog (S. cerevisiae)
|
|
ICT1
|
immature colon carcinoma transcript 1
|
|
IDH2
|
isocitrate dehydrogenase 2 (NADP+), mitochondrial
|
|
IDH3A
|
isocitrate dehydrogenase 3 (NAD+) alpha
|
|
IDH3B
|
isocitrate dehydrogenase 3 (NAD+) beta
|
|
IDH3G
|
isocitrate dehydrogenase 3 (NAD+) gamma
|
|
ISCU
|
iron-sulfur cluster assembly enzyme
|
|
IVD
|
isovaleryl-CoA dehydrogenase
|
|
KARS
|
lysyl-tRNA synthetase
|
|
LARS2
|
leucyl-tRNA synthetase 2, mitochondrial
|
|
LONP1
|
lon peptidase 1, mitochondrial
|
|
LRRK2
|
leucine-rich repeat kinase 2
|
|
LYRM4
|
LYR motif containing 4
|
|
LYRM7
|
LYR motif containing 7
|
|
MALSU1
|
mitochondrial assembly of ribosomal large subunit 1
|
|
MARS2
|
methionyl-tRNA synthetase 2, mitochondrial
|
|
MCCC1
|
methylcrotonoyl-CoA carboxylase 1 (alpha)
|
|
MCCC2
|
methylcrotonoyl-CoA carboxylase 2 (beta)
|
|
MCEE
|
methylmalonyl CoA epimerase
|
|
MCL1
|
myeloid cell leukemia 1
|
|
MDH2
|
malate dehydrogenase 2, NAD (mitochondrial)
|
|
ME2
|
malic enzyme 2, NAD(+)-dependent, mitochondrial
|
|
ME3
|
malic enzyme 3, NADP(+)-dependent, mitochondrial
|
|
METTL20
|
methyltransferase like 20
|
|
MIPEP
|
mitochondrial intermediate peptidase
|
|
MLYCD
|
malonyl-CoA decarboxylase
|
|
MMAA
|
methylmalonic aciduria (cobalamin deficiency) cblA type
|
|
MMAB
|
methylmalonic aciduria (cobalamin deficiency) cblB type
|
|
MRRF
|
mitochondrial ribosome recycling factor
|
|
MTERF1
|
mitochondrial transcription termination factor 1
|
|
MTERF2
|
mitochondrial transcription termination factor 2
|
|
MTG1
|
mitochondrial ribosome-associated GTPase 1
|
|
MTG2
|
mitochondrial ribosome-associated GTPase 2
|
|
MTRF1L
|
mitochondrial translational release factor 1-like
|
|
MUT
|
methylmalonyl CoA mutase
|
|
NAGS
|
N-acetylglutamate synthase
|
|
NARS2
|
asparaginyl-tRNA synthetase 2, mitochondrial (putative)
|
|
NDUFA10
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa
|
|
NDUFA9
|
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
|
|
NDUFAB1
|
NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa
|
|
NFS1
|
NFS1 cysteine desulfurase
|
|
NR3C1
|
nuclear receptor subfamily 3, group C, member 1 (glucocorticoid receptor)
|
|
NT5M
|
5',3'-nucleotidase, mitochondrial
|
|
NUDT1
|
nudix (nucleoside diphosphate linked moiety X)-type motif 1
|
|
NUDT9
|
nudix (nucleoside diphosphate linked moiety X)-type motif 9
|
|
OAT
|
ornithine aminotransferase
|
|
OGDH
|
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
|
|
OGDHL
|
oxoglutarate dehydrogenase-like
|
|
OTC
|
ornithine carbamoyltransferase
|
|
OXCT1
|
3-oxoacid CoA transferase 1
|
|
PABPC5
|
poly(A) binding protein, cytoplasmic 5
|
|
PAM16
|
presequence translocase-associated motor 16 homolog (S. cerevisiae)
|
|
PARG
|
poly (ADP-ribose) glycohydrolase
|
|
PARK7
|
parkinson protein 7
|
|
PARS2
|
prolyl-tRNA synthetase 2, mitochondrial (putative)
|
|
PC
|
pyruvate carboxylase
|
|
PCCA
|
propionyl CoA carboxylase, alpha polypeptide
|
|
PCCB
|
propionyl CoA carboxylase, beta polypeptide
|
|
PCK2
|
phosphoenolpyruvate carboxykinase 2 (mitochondrial)
|
|
PDE12
|
phosphodiesterase 12
|
|
PDE2A
|
phosphodiesterase 2A, cGMP-stimulated
|
|
PDHA1
|
pyruvate dehydrogenase (lipoamide) alpha 1
|
|
PDHA2
|
pyruvate dehydrogenase (lipoamide) alpha 2
|
|
PDHB
|
pyruvate dehydrogenase (lipoamide) beta
|
|
PDHX
|
pyruvate dehydrogenase complex, component X
|
|
PDK1
|
pyruvate dehydrogenase kinase, isozyme 1
|
|
PDK2
|
pyruvate dehydrogenase kinase, isozyme 2
|
|
PDK3
|
pyruvate dehydrogenase kinase, isozyme 3
|
|
PDK4
|
pyruvate dehydrogenase kinase, isozyme 4
|
|
PDP1
|
pyruvate dehyrogenase phosphatase catalytic subunit 1
|
|
PDP2
|
pyruvate dehyrogenase phosphatase catalytic subunit 2
|
|
PDPR
|
pyruvate dehydrogenase phosphatase regulatory subunit
|
|
PDSS1
|
prenyl (decaprenyl) diphosphate synthase, subunit 1
|
|
PDSS2
|
prenyl (decaprenyl) diphosphate synthase, subunit 2
|
|
PIN4
|
peptidylprolyl cis/trans isomerase, NIMA-interacting 4
|
|
PITRM1
|
pitrilysin metallopeptidase 1
|
|
PMPCA
|
peptidase (mitochondrial processing) alpha
|
|
PMPCB
|
peptidase (mitochondrial processing) beta
|
|
POLG2
|
polymerase (DNA directed), gamma 2, accessory subunit
|
|
POLRMT
|
polymerase (RNA) mitochondrial (DNA directed)
|
|
PPA2
|
pyrophosphatase (inorganic) 2
|
|
PPIF
|
peptidylprolyl isomerase F
|
|
PPM1K
|
protein phosphatase, Mg2+/Mn2+ dependent, 1K
|
|
PRDX1
|
peroxiredoxin 1
|
|
PRDX3
|
peroxiredoxin 3
|
|
PRDX5
|
peroxiredoxin 5
|
|
PRIMPOL
|
primase and polymerase (DNA-directed)
|
|
PRODH
|
proline dehydrogenase (oxidase) 1
|
|
PTCD1
|
pentatricopeptide repeat domain 1
|
|
PYCR1
|
pyrroline-5-carboxylate reductase 1
|
|
QARS
|
glutaminyl-tRNA synthetase
|
|
RAD51
|
RAD51 recombinase
|
|
RARS2
|
arginyl-tRNA synthetase 2, mitochondrial
|
|
REXO2
|
RNA exonuclease 2
|
|
SARDH
|
sarcosine dehydrogenase
|
|
SARS2
|
seryl-tRNA synthetase 2, mitochondrial
|
|
SDHAF1
|
succinate dehydrogenase complex assembly factor 1
|
|
SDHAF2
|
succinate dehydrogenase complex assembly factor 2
|
|
SDHAF3
|
succinate dehydrogenase complex assembly factor 3
|
|
SDHAF4
|
succinate dehydrogenase complex assembly factor 4
|
|
SHC1
|
SHC (Src homology 2 domain containing) transforming protein 1
|
|
SHMT2
|
serine hydroxymethyltransferase 2 (mitochondrial)
|
|
SIRT3
|
sirtuin 3
|
|
SIRT4
|
sirtuin 4
|
|
SIRT5
|
sirtuin 5
|
|
SOD1
|
superoxide dismutase 1, soluble
|
|
SOD2
|
superoxide dismutase 2, mitochondrial
|
|
SSBP1
|
single-stranded DNA binding protein 1, mitochondrial
|
|
SUCLA2
|
succinate-CoA ligase, ADP-forming, beta subunit
|
|
SUCLG1
|
succinate-CoA ligase, alpha subunit
|
|
SUCLG2
|
succinate-CoA ligase, GDP-forming, beta subunit
|
|
SUOX
|
sulfite oxidase
|
|
SUPV3L1
|
suppressor of var1, 3-like 1 (S. cerevisiae)
|
|
TARS2
|
threonyl-tRNA synthetase 2, mitochondrial (putative)
|
|
TDRD7
|
tudor domain containing 7
|
|
TEFM
|
transcription elongation factor, mitochondrial
|
|
TFAM
|
transcription factor A, mitochondrial
|
|
TFB1M
|
transcription factor B1, mitochondrial
|
|
TFB2M
|
transcription factor B2, mitochondrial
|
|
THEM5
|
thioesterase superfamily member 5
|
|
TIMM44
|
translocase of inner mitochondrial membrane 44 homolog (yeast)
|
|
TK2
|
thymidine kinase 2, mitochondrial
|
|
TMLHE
|
trimethyllysine hydroxylase, epsilon
|
|
TP53
|
tumor protein p53
|
|
TRAP1
|
TNF receptor-associated protein 1
|
|
TRMT5
|
tRNA methyltransferase 5
|
|
TST
|
thiosulfate sulfurtransferase (rhodanese)
|
|
TXN2
|
thioredoxin 2
|
|
TXNRD2
|
thioredoxin reductase 2
|
|
TYMS
|
thymidylate synthetase
|
|
UQCC2
|
ubiquinol-cytochrome c reductase complex assembly factor 2
|
|
WARS2
|
tryptophanyl tRNA synthetase 2, mitochondrial
|
|
YARS2
|
tyrosyl-tRNA synthetase 2, mitochondrial
|
