| Symbol |
Name |
|
AARS2
|
alanyl-tRNA synthetase 2, mitochondrial
|
|
ABCC8
|
ATP-binding cassette, sub-family C (CFTR/MRP), member 8
|
|
ABCD4
|
ATP-binding cassette, sub-family D (ALD), member 4
|
|
ACAD9
|
acyl-CoA dehydrogenase family, member 9
|
|
ACADM
|
acyl-CoA dehydrogenase, C-4 to C-12 straight chain
|
|
ACADS
|
acyl-CoA dehydrogenase, C-2 to C-3 short chain
|
|
ACAT1
|
acetyl-CoA acetyltransferase 1
|
|
ACAT2
|
acetyl-CoA acetyltransferase 2
|
|
ACSF3
|
acyl-CoA synthetase family member 3
|
|
ADAMTS13
|
ADAM metallopeptidase with thrombospondin type 1 motif, 13
|
|
ADCK3
|
aarF domain containing kinase 3
|
|
AGK
|
acylglycerol kinase
|
|
AGXT
|
alanine-glyoxylate aminotransferase
|
|
AIFM1
|
apoptosis-inducing factor, mitochondrion-associated, 1
|
|
ALDOB
|
aldolase B, fructose-bisphosphate
|
|
ARG1
|
arginase 1
|
|
ASL
|
argininosuccinate lyase
|
|
ASS1
|
argininosuccinate synthase 1
|
|
ATP5E
|
ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit
|
|
ATP6V0A4
|
ATPase, H+ transporting, lysosomal V0 subunit a4
|
|
ATP6V1B1
|
ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1
|
|
ATPAF2
|
ATP synthase mitochondrial F1 complex assembly factor 2
|
|
AUH
|
AU RNA binding protein/enoyl-CoA hydratase
|
|
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
|
|
BTD
|
biotinidase
|
|
C10ORF2
|
chromosome 10 open reading frame 2
|
|
C12ORF65
|
chromosome 12 open reading frame 65
|
|
CA2
|
carbonic anhydrase II
|
|
CA5A
|
carbonic anhydrase VA, mitochondrial
|
|
CLDN16
|
claudin 16
|
|
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)
|
|
CPT1A
|
carnitine palmitoyltransferase 1A (liver)
|
|
CTNS
|
cystinosin, lysosomal cystine transporter
|
|
CUL3
|
cullin 3
|
|
CYC1
|
cytochrome c-1
|
|
DBT
|
dihydrolipoamide branched chain transacylase E2
|
|
DECR1
|
2,4-dienoyl CoA reductase 1, mitochondrial
|
|
DGUOK
|
deoxyguanosine kinase
|
|
DLAT
|
dihydrolipoamide S-acetyltransferase
|
|
DLD
|
dihydrolipoamide dehydrogenase
|
|
DNM1L
|
dynamin 1-like
|
|
DPYS
|
dihydropyrimidinase
|
|
EARS2
|
glutamyl-tRNA synthetase 2, mitochondrial
|
|
EHHADH
|
enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase
|
|
ELAC2
|
elaC ribonuclease Z 2
|
|
EPG5
|
ectopic P-granules autophagy protein 5 homolog (C. elegans)
|
|
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
|
|
FASTKD2
|
FAST kinase domains 2
|
|
FBP1
|
fructose-1,6-bisphosphatase 1
|
|
FBXL4
|
F-box and leucine-rich repeat protein 4
|
|
FH
|
fumarate hydratase
|
|
FN1
|
fibronectin 1
|
|
FOXRED1
|
FAD-dependent oxidoreductase domain containing 1
|
|
G6PC
|
glucose-6-phosphatase, catalytic subunit
|
|
GALT
|
galactose-1-phosphate uridylyltransferase
|
|
GCDH
|
glutaryl-CoA dehydrogenase
|
|
GCK
|
glucokinase (hexokinase 4)
|
|
GFM1
|
G elongation factor, mitochondrial 1
|
|
GK
|
glycerol kinase
|
|
GLYCTK
|
glycerate kinase
|
|
GSS
|
glutathione synthetase
|
|
GYS2
|
glycogen synthase 2 (liver)
|
|
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
|
|
HCFC1
|
host cell factor C1
|
|
HLCS
|
holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
|
|
HMGCL
|
3-hydroxymethyl-3-methylglutaryl-CoA lyase
|
|
HPD
|
4-hydroxyphenylpyruvate dioxygenase
|
|
HSD17B10
|
hydroxysteroid (17-beta) dehydrogenase 10
|
|
IBA57
|
IBA57, iron-sulfur cluster assembly homolog (S. cerevisiae)
|
|
INS
|
insulin
|
|
INSR
|
insulin receptor
|
|
INVS
|
inversin
|
|
ISCU
|
iron-sulfur cluster assembly enzyme
|
|
IVD
|
isovaleryl-CoA dehydrogenase
|
|
JAG1
|
jagged 1
|
|
KCNJ11
|
potassium channel, inwardly rectifying subfamily J, member 11
|
|
KCNJ5
|
potassium channel, inwardly rectifying subfamily J, member 5
|
|
KLHL3
|
kelch-like family member 3
|
|
LCT
|
lactase
|
|
LDHA
|
lactate dehydrogenase A
|
|
LIAS
|
lipoic acid synthetase
|
|
LMBRD1
|
LMBR1 domain containing 1
|
|
LRPPRC
|
leucine-rich pentatricopeptide repeat containing
|
|
LYRM4
|
LYR motif containing 4
|
|
LYRM7
|
LYR motif containing 7
|
|
MCCC1
|
methylcrotonoyl-CoA carboxylase 1 (alpha)
|
|
MCCC2
|
methylcrotonoyl-CoA carboxylase 2 (beta)
|
|
MCEE
|
methylmalonyl CoA epimerase
|
|
MLYCD
|
malonyl-CoA decarboxylase
|
|
MMAA
|
methylmalonic aciduria (cobalamin deficiency) cblA type
|
|
MMAB
|
methylmalonic aciduria (cobalamin deficiency) cblB type
|
|
MMACHC
|
methylmalonic aciduria (cobalamin deficiency) cblC type, with homocystinuria
|
|
MMADHC
|
methylmalonic aciduria (cobalamin deficiency) cblD type, with homocystinuria
|
|
MPC1
|
mitochondrial pyruvate carrier 1
|
|
MPV17
|
MpV17 mitochondrial inner membrane protein
|
|
MRPL3
|
mitochondrial ribosomal protein L3
|
|
MRPL44
|
mitochondrial ribosomal protein L44
|
|
MRPS16
|
mitochondrial ribosomal protein S16
|
|
MRPS22
|
mitochondrial ribosomal protein S22
|
|
MTFMT
|
mitochondrial methionyl-tRNA formyltransferase
|
|
MTO1
|
mitochondrial tRNA translation optimization 1
|
|
MUT
|
methylmalonyl CoA mutase
|
|
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
|
|
NEUROG3
|
neurogenin 3
|
|
NFU1
|
NFU1 iron-sulfur cluster scaffold
|
|
NOTCH2
|
notch 2
|
|
NR3C2
|
nuclear receptor subfamily 3, group C, member 2
|
|
NUBPL
|
nucleotide binding protein-like
|
|
OCRL
|
oculocerebrorenal syndrome of Lowe
|
|
OGDH
|
oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
|
|
OXCT1
|
3-oxoacid CoA transferase 1
|
|
PAH
|
phenylalanine hydroxylase
|
|
PAX4
|
paired box 4
|
|
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
|
|
PDSS1
|
prenyl (decaprenyl) diphosphate synthase, subunit 1
|
|
PDSS2
|
prenyl (decaprenyl) diphosphate synthase, subunit 2
|
|
PET100
|
PET100 homolog (S. cerevisiae)
|
|
PHKG2
|
phosphorylase kinase, gamma 2 (testis)
|
|
PNPO
|
pyridoxamine 5'-phosphate oxidase
|
|
POLG
|
polymerase (DNA directed), gamma
|
|
POLG2
|
polymerase (DNA directed), gamma 2, accessory subunit
|
|
PUS1
|
pseudouridylate synthase 1
|
|
RARS2
|
arginyl-tRNA synthetase 2, mitochondrial
|
|
RMND1
|
required for meiotic nuclear division 1 homolog (S. cerevisiae)
|
|
RRM2B
|
ribonucleotide reductase M2 B (TP53 inducible)
|
|
RYR1
|
ryanodine receptor 1 (skeletal)
|
|
SCNN1A
|
sodium channel, non voltage gated 1 alpha subunit
|
|
SCNN1B
|
sodium channel, non voltage gated 1 beta subunit
|
|
SCNN1G
|
sodium channel, non voltage gated 1 gamma subunit
|
|
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
|
|
SLC2A2
|
solute carrier family 2 (facilitated glucose transporter), member 2
|
|
SLC37A4
|
solute carrier family 37 (glucose-6-phosphate transporter), member 4
|
|
SLC4A1
|
solute carrier family 4 (anion exchanger), member 1 (Diego blood group)
|
|
SLC4A4
|
solute carrier family 4 (sodium bicarbonate cotransporter), member 4
|
|
SLC5A1
|
solute carrier family 5 (sodium/glucose cotransporter), member 1
|
|
SLC7A7
|
solute carrier family 7 (amino acid transporter light chain, y+L system), member 7
|
|
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
|
|
TARS2
|
threonyl-tRNA synthetase 2, mitochondrial (putative)
|
|
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
|
|
UMPS
|
uridine monophosphate synthetase
|
|
UQCC2
|
ubiquinol-cytochrome c reductase complex assembly factor 2
|
|
UQCRB
|
ubiquinol-cytochrome c reductase binding protein
|
|
UQCRC2
|
ubiquinol-cytochrome c reductase core protein II
|
|
UQCRQ
|
ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa
|
|
VIPAS39
|
VPS33B interacting protein, apical-basolateral polarity regulator, spe-39 homolog
|
|
VPS33B
|
vacuolar protein sorting 33 homolog B (yeast)
|
|
VTI1A
|
vesicle transport through interaction with t-SNAREs 1A
|
|
WNK1
|
WNK lysine deficient protein kinase 1
|
|
WNK4
|
WNK lysine deficient protein kinase 4
|
|
YARS2
|
tyrosyl-tRNA synthetase 2, mitochondrial
|
