Source Id |
NCBI (Entrez) Gene Id |
Gene Symbol |
Gene Description |
100131801 |
100131801 |
PET100 |
PET100 cytochrome c oxidase chaperon... |
100303755 |
100303755 |
PET117 |
PET117 cytochrome c oxidase chaperon... |
10059 |
10059 |
DNM1L |
dynamin 1 like [Source:HGNC Symbol;A... |
10102 |
10102 |
TSFM |
Ts translation elongation factor, mi... |
10128 |
10128 |
LRPPRC |
leucine rich pentatricopeptide repea... |
1020 |
1020 |
CDK5 |
cyclin dependent kinase 5 [Source:HG... |
10352 |
10352 |
WARS2 |
tryptophanyl tRNA synthetase 2, mito... |
10531 |
10531 |
PITRM1 |
pitrilysin metallopeptidase 1 [Sourc... |
10667 |
10667 |
FARS2 |
phenylalanyl-tRNA synthetase 2, mito... |
10730 |
10730 |
YME1L1 |
YME1 like 1 ATPase [Source:HGNC Symb... |
11019 |
11019 |
LIAS |
lipoic acid synthetase [Source:HGNC ... |
11093 |
11093 |
ADAMTS13 |
ADAM metallopeptidase with thrombosp... |
11212 |
11212 |
PLPBP |
pyridoxal phosphate binding protein ... |
11222 |
11222 |
MRPL3 |
mitochondrial ribosomal protein L3 [... |
11232 |
11232 |
POLG2 |
DNA polymerase gamma 2, accessory su... |
112812 |
112812 |
FDX2 |
ferredoxin 2 [Source:HGNC Symbol;Acc... |
115286 |
115286 |
SLC25A26 |
solute carrier family 25 member 26 [... |
116228 |
116228 |
COX20 |
cytochrome c oxidase assembly factor... |
119559 |
119559 |
SFXN4 |
sideroflexin 4 [Source:HGNC Symbol;A... |
123263 |
123263 |
MTFMT |
mitochondrial methionyl-tRNA formylt... |
124454 |
124454 |
EARS2 |
glutamyl-tRNA synthetase 2, mitochon... |
125988 |
125988 |
MICOS13 |
mitochondrial contact site and crist... |
126328 |
126328 |
NDUFA11 |
NADH:ubiquinone oxidoreductase subun... |
128240 |
128240 |
NAXE |
NAD(P)HX epimerase [Source:HGNC Symb... |
128989 |
128989 |
TANGO2 |
transport and golgi organization 2 h... |
131118 |
131118 |
DNAJC19 |
DnaJ heat shock protein family (Hsp4... |
1317 |
1317 |
SLC31A1 |
solute carrier family 31 member 1 [S... |
1327 |
1327 |
COX4I1 |
cytochrome c oxidase subunit 4I1 [So... |
133686 |
133686 |
NADK2 |
NAD kinase 2, mitochondrial [Source:... |
1339 |
1339 |
COX6A2 |
cytochrome c oxidase subunit 6A2 [So... |
1340 |
1340 |
COX6B1 |
cytochrome c oxidase subunit 6B1 [So... |
1351 |
1351 |
COX8A |
cytochrome c oxidase subunit 8A [Sou... |
1352 |
1352 |
COX10 |
cytochrome c oxidase assembly factor... |
1353 |
1353 |
COX11 |
cytochrome c oxidase copper chaperon... |
137682 |
137682 |
NDUFAF6 |
NADH:ubiquinone oxidoreductase compl... |
1384 |
1384 |
CRAT |
carnitine O-acetyltransferase [Sourc... |
1468 |
1468 |
SLC25A10 |
solute carrier family 25 member 10 [... |
1537 |
1537 |
CYC1 |
cytochrome c1 [Source:HGNC Symbol;Ac... |
1593 |
1593 |
CYP27A1 |
cytochrome P450 family 27 subfamily ... |
1716 |
1716 |
DGUOK |
deoxyguanosine kinase [Source:HGNC S... |
1738 |
1738 |
DLD |
dihydrolipoamide dehydrogenase [Sour... |
1841 |
1841 |
DTYMK |
deoxythymidylate kinase [Source:HGNC... |
1892 |
1892 |
ECHS1 |
enoyl-CoA hydratase, short chain 1 [... |
22868 |
22868 |
FASTKD2 |
FAST kinase domains 2 [Source:HGNC S... |
23395 |
23395 |
LARS2 |
leucyl-tRNA synthetase 2, mitochondr... |
23479 |
23479 |
ISCU |
iron-sulfur cluster assembly enzyme ... |
23590 |
23590 |
PDSS1 |
decaprenyl diphosphate synthase subu... |
246243 |
246243 |
RNASEH1 |
ribonuclease H1 [Source:HGNC Symbol;... |
25821 |
25821 |
MTO1 |
mitochondrial tRNA translation optim... |
25828 |
25828 |
TXN2 |
thioredoxin 2 [Source:HGNC Symbol;Ac... |
26235 |
26235 |
FBXL4 |
F-box and leucine rich repeat protei... |
26275 |
26275 |
HIBCH |
3-hydroxyisobutyryl-CoA hydrolase [S... |
270 |
270 |
AMPD1 |
adenosine monophosphate deaminase 1 ... |
27010 |
27010 |
TPK1 |
thiamin pyrophosphokinase 1 [Source:... |
27089 |
27089 |
UQCRQ |
ubiquinol-cytochrome c reductase com... |
271 |
271 |
AMPD2 |
adenosine monophosphate deaminase 2 ... |
2710 |
2710 |
GK |
glycerol kinase [Source:HGNC Symbol;... |
272 |
272 |
AMPD3 |
adenosine monophosphate deaminase 3 ... |
27247 |
27247 |
NFU1 |
NFU1 iron-sulfur cluster scaffold [S... |
27429 |
27429 |
HTRA2 |
HtrA serine peptidase 2 [Source:HGNC... |
2806 |
2806 |
GOT2 |
glutamic-oxaloacetic transaminase 2 ... |
284439 |
284439 |
SLC25A42 |
solute carrier family 25 member 42 [... |
28957 |
28957 |
MRPS28 |
mitochondrial ribosomal protein S28 ... |
28976 |
28976 |
ACAD9 |
acyl-CoA dehydrogenase family member... |
29078 |
29078 |
NDUFAF4 |
NADH:ubiquinone oxidoreductase compl... |
291 |
291 |
SLC25A4 |
solute carrier family 25 member 4 [S... |
29928 |
29928 |
TIMM22 |
translocase of inner mitochondrial m... |
2998 |
2998 |
GYS2 |
glycogen synthase 2 [Source:HGNC Sym... |
3028 |
3028 |
HSD17B10 |
hydroxysteroid 17-beta dehydrogenase... |
3032 |
3032 |
HADHB |
hydroxyacyl-CoA dehydrogenase trifun... |
31 |
31 |
ACACA |
acetyl-CoA carboxylase alpha [Source... |
3155 |
3155 |
HMGCL |
3-hydroxy-3-methylglutaryl-CoA lyase... |
3329 |
3329 |
HSPD1 |
heat shock protein family D (Hsp60) ... |
3735 |
3735 |
KARS1 |
lysyl-tRNA synthetase 1 [Source:HGNC... |
38 |
38 |
ACAT1 |
acetyl-CoA acetyltransferase 1 [Sour... |
387787 |
387787 |
LIPT2 |
lipoyl(octanoyl) transferase 2 [Sour... |
39 |
39 |
ACAT2 |
acetyl-CoA acetyltransferase 2 [Sour... |
3939 |
3939 |
LDHA |
lactate dehydrogenase A [Source:HGNC... |
3954 |
3954 |
LETM1 |
leucine zipper and EF-hand containin... |
400916 |
400916 |
CHCHD10 |
coiled-coil-helix-coiled-coil-helix ... |
4191 |
4191 |
MDH2 |
malate dehydrogenase 2 [Source:HGNC ... |
4204 |
4204 |
MECP2 |
methyl-CpG binding protein 2 [Source... |
4285 |
4285 |
MIPEP |
mitochondrial intermediate peptidase... |
4329 |
4329 |
ALDH6A1 |
aldehyde dehydrogenase 6 family memb... |
4508 |
4508 |
MT-ATP6 |
mitochondrially encoded ATP synthase... |
4512 |
4512 |
MT-CO1 |
mitochondrially encoded cytochrome c... |
4513 |
4513 |
MT-CO2 |
mitochondrially encoded cytochrome c... |
4514 |
4514 |
MT-CO3 |
mitochondrially encoded cytochrome c... |
4535 |
4535 |
MT-ND1 |
mitochondrially encoded NADH:ubiquin... |
4536 |
4536 |
MT-ND2 |
mitochondrially encoded NADH:ubiquin... |
4537 |
4537 |
MT-ND3 |
mitochondrially encoded NADH:ubiquin... |
4538 |
4538 |
MT-ND4 |
mitochondrially encoded NADH:ubiquin... |
4540 |
4540 |
MT-ND5 |
mitochondrially encoded NADH:ubiquin... |
4541 |
4541 |
MT-ND6 |
mitochondrially encoded NADH:ubiquin... |
4558 |
4558 |
MT-TF |
mitochondrially encoded tRNA-Phe (UU... |
4564 |
4564 |
MT-TH |
mitochondrially encoded tRNA-His (CA... |
4565 |
4565 |
MT-TI |
mitochondrially encoded tRNA-Ile (AU... |
4566 |
4566 |
MT-TK |
mitochondrially encoded tRNA-Lys (AA... |
4567 |
4567 |
MT-TL1 |
mitochondrially encoded tRNA-Leu (UU... |
4568 |
4568 |
MT-TL2 |
mitochondrially encoded tRNA-Leu (CU... |
4570 |
4570 |
MT-TN |
mitochondrially encoded tRNA-Asn (AA... |
4571 |
4571 |
MT-TP |
mitochondrially encoded tRNA-Pro (CC... |
4572 |
4572 |
MT-TQ |
mitochondrially encoded tRNA-Gln (CA... |
4574 |
4574 |
MT-TS1 |
mitochondrially encoded tRNA-Ser (UC... |
4575 |
4575 |
MT-TS2 |
mitochondrially encoded tRNA-Ser (AG... |
4577 |
4577 |
MT-TV |
mitochondrially encoded tRNA-Val (GU... |
4578 |
4578 |
MT-TW |
mitochondrially encoded tRNA-Trp (UG... |
4694 |
4694 |
NDUFA1 |
NADH:ubiquinone oxidoreductase subun... |
4700 |
4700 |
NDUFA6 |
NADH:ubiquinone oxidoreductase subun... |
4702 |
4702 |
NDUFA8 |
NADH:ubiquinone oxidoreductase subun... |
4704 |
4704 |
NDUFA9 |
NADH:ubiquinone oxidoreductase subun... |
4714 |
4714 |
NDUFB8 |
NADH:ubiquinone oxidoreductase subun... |
4715 |
4715 |
NDUFB9 |
NADH:ubiquinone oxidoreductase subun... |
4718 |
4718 |
NDUFC2 |
NADH:ubiquinone oxidoreductase subun... |
4719 |
4719 |
NDUFS1 |
NADH:ubiquinone oxidoreductase core ... |
4720 |
4720 |
NDUFS2 |
NADH:ubiquinone oxidoreductase core ... |
4722 |
4722 |
NDUFS3 |
NADH:ubiquinone oxidoreductase core ... |
4723 |
4723 |
NDUFV1 |
NADH:ubiquinone oxidoreductase core ... |
4728 |
4728 |
NDUFS8 |
NADH:ubiquinone oxidoreductase core ... |
4952 |
4952 |
OCRL |
OCRL inositol polyphosphate-5-phosph... |
4967 |
4967 |
OGDH |
oxoglutarate dehydrogenase [Source:H... |
4976 |
4976 |
OPA1 |
OPA1 mitochondrial dynamin like GTPa... |
498 |
498 |
ATP5F1A |
ATP synthase F1 subunit alpha [Sourc... |
50484 |
50484 |
RRM2B |
ribonucleotide reductase regulatory ... |
50640 |
50640 |
PNPLA8 |
patatin like phospholipase domain co... |
5091 |
5091 |
PC |
pyruvate carboxylase [Source:HGNC Sy... |
51021 |
51021 |
MRPS16 |
mitochondrial ribosomal protein S16 ... |
51067 |
51067 |
YARS2 |
tyrosyl-tRNA synthetase 2 [Source:HG... |
51079 |
51079 |
NDUFA13 |
NADH:ubiquinone oxidoreductase subun... |
51081 |
51081 |
MRPS7 |
mitochondrial ribosomal protein S7 [... |
51103 |
51103 |
NDUFAF1 |
NADH:ubiquinone oxidoreductase compl... |
51116 |
51116 |
MRPS2 |
mitochondrial ribosomal protein S2 [... |
51117 |
51117 |
COQ4 |
coenzyme Q4 [Source:HGNC Symbol;Acc:... |
51218 |
51218 |
GLRX5 |
glutaredoxin 5 [Source:HGNC Symbol;A... |
5160 |
5160 |
PDHA1 |
pyruvate dehydrogenase E1 subunit al... |
51601 |
51601 |
LIPT1 |
lipoyltransferase 1 [Source:HGNC Sym... |
51660 |
51660 |
MPC1 |
mitochondrial pyruvate carrier 1 [So... |
51733 |
51733 |
UPB1 |
beta-ureidopropionase 1 [Source:HGNC... |
5250 |
5250 |
SLC25A3 |
solute carrier family 25 member 3 [S... |
5261 |
5261 |
PHKG2 |
phosphorylase kinase catalytic subun... |
54148 |
54148 |
MRPL39 |
mitochondrial ribosomal protein L39 ... |
5428 |
5428 |
POLG |
DNA polymerase gamma, catalytic subu... |
5442 |
5442 |
POLRMT |
RNA polymerase mitochondrial [Source... |
54704 |
54704 |
PDP1 |
pyruvate dehydrogenase phosphatase c... |
54931 |
54931 |
TRMT10C |
tRNA methyltransferase 10C, mitochon... |
54938 |
54938 |
SARS2 |
seryl-tRNA synthetase 2, mitochondri... |
54968 |
54968 |
TMEM70 |
transmembrane protein 70 [Source:HGN... |
55005 |
55005 |
RMND1 |
required for meiotic nuclear divisio... |
55157 |
55157 |
DARS2 |
aspartyl-tRNA synthetase 2, mitochon... |
55163 |
55163 |
PNPO |
pyridoxamine 5'-phosphate oxidase [S... |
55210 |
55210 |
ATAD3A |
ATPase family AAA domain containing ... |
55687 |
55687 |
TRMU |
tRNA mitochondrial 2-thiouridylase [... |
55739 |
55739 |
NAXD |
NAD(P)HX dehydratase [Source:HGNC Sy... |
55750 |
55750 |
AGK |
acylglycerol kinase [Source:HGNC Sym... |
55768 |
55768 |
NGLY1 |
N-glycanase 1 [Source:HGNC Symbol;Ac... |
55863 |
55863 |
TMEM126B |
transmembrane protein 126B [Source:H... |
55967 |
55967 |
NDUFA12 |
NADH:ubiquinone oxidoreductase subun... |
56652 |
56652 |
TWNK |
twinkle mtDNA helicase [Source:HGNC ... |
56945 |
56945 |
MRPS22 |
mitochondrial ribosomal protein S22 ... |
56997 |
56997 |
COQ8A |
coenzyme Q8A [Source:HGNC Symbol;Acc... |
57017 |
57017 |
COQ9 |
coenzyme Q9 [Source:HGNC Symbol;Acc:... |
57038 |
57038 |
RARS2 |
arginyl-tRNA synthetase 2, mitochond... |
57107 |
57107 |
PDSS2 |
decaprenyl diphosphate synthase subu... |
57505 |
57505 |
AARS2 |
alanyl-tRNA synthetase 2, mitochondr... |
57570 |
57570 |
TRMT5 |
tRNA methyltransferase 5 [Source:HGN... |
5836 |
5836 |
PYGL |
glycogen phosphorylase L [Source:HGN... |
5917 |
5917 |
RARS1 |
arginyl-tRNA synthetase 1 [Source:HG... |
617 |
617 |
BCS1L |
BCS1 homolog, ubiquinol-cytochrome c... |
6182 |
6182 |
MRPL12 |
mitochondrial ribosomal protein L12 ... |
6341 |
6341 |
SCO1 |
synthesis of cytochrome C oxidase 1 ... |
6389 |
6389 |
SDHA |
succinate dehydrogenase complex flav... |
63899 |
63899 |
NSUN3 |
NOP2/Sun RNA methyltransferase 3 [So... |
6390 |
6390 |
SDHB |
succinate dehydrogenase complex iron... |
63931 |
63931 |
MRPS14 |
mitochondrial ribosomal protein S14 ... |
64121 |
64121 |
RRAGC |
Ras related GTP binding C [Source:HG... |
65080 |
65080 |
MRPL44 |
mitochondrial ribosomal protein L44 ... |
65993 |
65993 |
MRPS34 |
mitochondrial ribosomal protein S34 ... |
6647 |
6647 |
SOD1 |
superoxide dismutase 1 [Source:HGNC ... |
6773 |
6773 |
STAT2 |
signal transducer and activator of t... |
6834 |
6834 |
SURF1 |
SURF1 cytochrome c oxidase assembly ... |
708 |
708 |
C1QBP |
complement C1q binding protein [Sour... |
7084 |
7084 |
TK2 |
thymidine kinase 2 [Source:HGNC Symb... |
7284 |
7284 |
TUFM |
Tu translation elongation factor, mi... |
7381 |
7381 |
UQCRB |
ubiquinol-cytochrome c reductase bin... |
7385 |
7385 |
UQCRC2 |
ubiquinol-cytochrome c reductase cor... |
7388 |
7388 |
UQCRH |
ubiquinol-cytochrome c reductase hin... |
763 |
763 |
CA5A |
carbonic anhydrase 5A [Source:HGNC S... |
790955 |
790955 |
UQCC3 |
ubiquinol-cytochrome c reductase com... |
79587 |
79587 |
CARS2 |
cysteinyl-tRNA synthetase 2, mitocho... |
79717 |
79717 |
PPCS |
phosphopantothenoylcysteine syntheta... |
79731 |
79731 |
NARS2 |
asparaginyl-tRNA synthetase 2, mitoc... |
79736 |
79736 |
TEFM |
transcription elongation factor, mit... |
79989 |
79989 |
IFT56 |
intraflagellar transport 56 [Source:... |
80222 |
80222 |
TARS2 |
threonyl-tRNA synthetase 2, mitochon... |
80324 |
80324 |
PUS1 |
pseudouridine synthase 1 [Source:HGN... |
8050 |
8050 |
PDHX |
pyruvate dehydrogenase complex compo... |
81570 |
81570 |
CLPB |
ClpB family mitochondrial disaggrega... |
81689 |
81689 |
ISCA1 |
iron-sulfur cluster assembly 1 [Sour... |
83548 |
83548 |
COG3 |
component of oligomeric golgi comple... |
84300 |
84300 |
UQCC2 |
ubiquinol-cytochrome c reductase com... |
84340 |
84340 |
GFM2 |
GTP dependent ribosome recycling fac... |
84705 |
84705 |
GTPBP3 |
GTP binding protein 3, mitochondrial... |
84842 |
84842 |
HPDL |
4-hydroxyphenylpyruvate dioxygenase ... |
8492 |
8492 |
PRSS12 |
serine protease 12 [Source:HGNC Symb... |
84947 |
84947 |
SERAC1 |
serine active site containing 1 [Sou... |
84987 |
84987 |
COX14 |
cytochrome c oxidase assembly factor... |
85476 |
85476 |
GFM1 |
G elongation factor mitochondrial 1 ... |
8565 |
8565 |
YARS1 |
tyrosyl-tRNA synthetase 1 [Source:HG... |
8604 |
8604 |
SLC25A12 |
solute carrier family 25 member 12 [... |
87178 |
87178 |
PNPT1 |
polyribonucleotide nucleotidyltransf... |
8802 |
8802 |
SUCLG1 |
succinate-CoA ligase GDP/ADP-forming... |
8803 |
8803 |
SUCLA2 |
succinate-CoA ligase ADP-forming sub... |
8867 |
8867 |
SYNJ1 |
synaptojanin 1 [Source:HGNC Symbol;A... |
90161 |
90161 |
HS6ST2 |
heparan sulfate 6-O-sulfotransferase... |
9054 |
9054 |
NFS1 |
NFS1 cysteine desulfurase [Source:HG... |
9056 |
9056 |
SLC7A7 |
solute carrier family 7 member 7 [So... |
90624 |
90624 |
LYRM7 |
LYR motif containing 7 [Source:HGNC ... |
9131 |
9131 |
AIFM1 |
apoptosis inducing factor mitochondr... |
91574 |
91574 |
MTRFR |
mitochondrial translation release fa... |
91647 |
91647 |
ATPAF2 |
ATP synthase mitochondrial F1 comple... |
92609 |
92609 |
TIMM50 |
translocase of inner mitochondrial m... |
9361 |
9361 |
LONP1 |
lon peptidase 1, mitochondrial [Sour... |
9377 |
9377 |
COX5A |
cytochrome c oxidase subunit 5A [Sou... |
9512 |
9512 |
PMPCB |
peptidase, mitochondrial processing ... |
9692 |
9692 |
PRORP |
protein only RNase P catalytic subun... |
9997 |
9997 |
SCO2 |
synthesis of cytochrome C oxidase 2 ... |