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. Author manuscript; available in PMC: 2016 Apr 1.
Published in final edited form as: Mol Genet Metab. 2015 Mar 5;114(4):570–579. doi: 10.1016/j.ymgme.2015.02.007

Table 3.

Significant abnormalities of proteins involved in mitochondrial function

ABCB71 ATP-binding cassette, sub-family B (MDR/TAP), member 7
ABCD31 ATP-binding cassette, sub-family D (ALD), member 3
ABCE1 ATP-binding cassette, sub-family E (OABP), member 1
ABHD112 abhydrolase domain containing 11
ACACA1 acetyl-coenzyme A carboxylase alpha
ACAD91 acyl-Coenzyme A dehydrogenase family, member 9
ACLY1 ATP citrate lyase
ACO2 aconitase 2, mitochondrial
ACSL11 acyl-CoA synthetase long-chain family member 1
ADSL adenylosuccinate lyase
AGPS1 alkylglycerone phosphate synthase
ALDH3A2 aldehyde dehydrogenase 3 family, member A2
ARMC10 armadillo repeat containing 10
ASL argininosuccinate lyase
ATAD11 ATPase family, AAA domain containing 1
ATIC 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase
ATP5B1,2 ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide
ATP5C12 ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1
ATP5D2 ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit
ATP5H2 ATP synthase, H+ transporting, mitochondrial F0 complex, subunit d
ATP5I2 ATP synthase, H+ transporting, mitochondrial F0 complex, subunit E
ATP6V1E12 ATPase, H+ transporting, lysosomal V1 subunit E1
ATPIF12 ATPase inhibitory factor 1; similar to ATPase inhibitor, mitochondrial precursor
BAX Bcl2-associated X protein
BCAT2 branched chain aminotransferase 2, mitochondrial
BOLA12 bolA homolog 1 (E. coli)
C1QBP2 complement component 1, q subcomponent binding protein
CCT71 chaperonin containing Tcp1, subunit 7 (eta)
CDS2 CDP-diacylglycerol synthase (phosphatidate cytidylyltransferase) 2
CHCHD32 coiled-coil-helix-coiled-coil-helix domain containing 3
CLIC4 chloride intracellular channel 4 (mitochondrial)
CLPB1 ClpB caseinolytic peptidase B homolog (E. coli)
CLTC clathrin, heavy chain (Hc)
CLU2 clusterin
COQ92 coenzyme Q9 homolog (S. cerevisiae)
COX2 COXII
COX4I2 cytochrome c oxidase subunit IV isoform 2
COX5A cytochrome c oxidase, subunit Va
COX5B2 cytochrome c oxidase subunit Vb
CPT1A carnitine palmitoyltransferase 1a, liver
CS citrate synthase; similar to citrate synthase
CTPS21 CTP synthase II
CYB5A cytochrome b5 type A (microsomal)
CYB5R1 cytochrome b5 reductase 1
CYB5R31 cytochrome b5 reductase 3
DBI2 diazepam binding inhibitor (GABA receptor modulator)
DIABLO2 diablo homolog (Drosophila)
DNM1L1 dynamin 1-like
DRG21 developmentally regulated GTP binding protein 2
ETFDH electron-transferring-flavoprotein dehydrogenase
FDPS farnesyl diphosphate synthase (farnesyl pyrophosphate synthetase, dimethylallyltranstransferase, geranyltranstransferase)
FKBP8 FK506 binding protein 8, 38kDa
FXN2 frataxin
GARS1 glycyl-tRNA synthetase
GCSH2 glycine cleavage system protein H (aminomethyl carrier)
GM2A2 GM2 ganglioside activator
GOT2 glutamic-oxaloacetic transaminase 2, mitochondrial (aspartate aminotransferase 2)
GPD1 glycerol-3-phosphate dehydrogenase 1 (soluble)
GPD2 glycerol-3-phosphate dehydrogenase 2, mitochondrial
GRPEL11,2 GrpE-like 1, mitochondrial
HADH2 hydroxyacyl-Coenzyme A dehydrogenase
HADHA hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
HAGH2 hydroxyacyl glutathione hydrolase
HINT22 histidine triad nucleotide binding protein 2
HK11 hexokinase 1
HK21 hexokinase 2
HSP90AB11 heat shock protein 90kDa alpha (cytosolic), class B member 1
HSPD11,2 60 kDa heat shock protein 1 (chaperonin)
HSPE11,2 heat shock protein 1 (chaperonin 10)
IARS2 isoleucyl-tRNA synthetase 2, mitochondrial
IDE1 insulin degrading enzyme
ISCU2 iron-sulfur cluster scaffold homolog (E. coli)
KRT5 keratin 5
LDHA lactate dehydrogenase A
LETM1 leucine zipper-EF-hand containing transmembrane protein 1
LOC606294 hypothetical protein LOC606294
LOC6888692 cytochrome c oxidase, subunit VIb polypeptide 1
LONP11 lon peptidase 1, mitochondrial
LRPPRC leucine-rich PPR-motif containing
LRRC59 leucine rich repeat containing 59
MAOA1 monoamine oxidase A
ME1 malic enzyme 1, NADP(+)-dependent, cytosolic
MGEA5 meningioma expressed antigen 5 (hyaluronidase)
MGST1 microsomal glutathione S-transferase 1
MRPL12 mitochondrial ribosomal protein L1
MRPL122 mitochondrial ribosomal protein L12
MRPL232 mitochondrial ribosomal protein L23
MRPL492 mitochondrial ribosomal protein L49
MRPS232 mitochondrial ribosomal protein S23
MRPS362 mitochondrial ribosomal protein S36
MTCH1 mitochondrial carrier homolog 1 (C. elegans)
MTCH2 mitochondrial carrier homolog 2 (C. elegans)
MTHFD11 methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1, methenyltetrahydrofolate cyclohydrolase, formyltetrahydrofolate synthetase
MTHFD1L1 methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like
NARS1 asparaginyl-tRNA synthetase
NDUFA22 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2
NDUFAF42 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 4
NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1
NDUFS2 NADH dehydrogenase (ubiquinone) Fe-S protein 2
NDUFS7 NADH dehydrogenase (ubiquinone) Fe-S protein 7
NDUFS82 NADH dehydrogenase (ubiquinone) Fe-S protein 8
NDUFV1 NADH dehydrogenase (ubiquinone) flavoprotein 1
NLN neurolysin (metallopeptidase M3 family)
NME31 non-metastatic cells 3, protein expressed in
NNT nicotinamide nucleotide transhydrogenase
NRD1 nardilysin 1 (N-arginine dibasic convertase)
OAT ornithine aminotransferase (gyrate atrophy)
OGDH oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
OPA11 optic atrophy 1 homolog (human)
PARK72 Parkinson disease (autosomal recessive, early onset) 7; similar to DJ-1
PC1 pyruvate carboxylase
PCK21 phosphoenolpyruvate carboxykinase 2 (mitochondrial)
PDE12 phosphodiesterase 12
PEBP11,2 phosphatidylethanolamine binding protein 1
PHB2 prohibitin
PHB22 prohibitin 2
PICK1 protein interacting with PRKCA 1
POR1 P450 (cytochrome) oxidoreductase
PPP1CC protein phosphatase 1, catalytic subunit, gamma isoform
PRDX62 peroxiredoxin 6
PRKACA1 protein kinase, cAMP-dependent, catalytic, alpha
PRKCD1 protein kinase C, delta
PSAP2 prosaposin
PTCD3 Pentatricopeptide repeat domain 3
PTPN11 protein tyrosine phosphatase, non-receptor type 11
QARS1 glutaminyl-tRNA synthetase
QDPR2 quinoid dihydropteridine reductase
RAB11B1 RAB11B, member RAS oncogene family
RAB351 RAB35, member RAS oncogene family
RAB3D1 RAB3D, member RAS oncogene family
RARS1 arginyl-tRNA synthetase
RGD13069172 similar to RIKEN cDNA 2900010M23; hypothetical protein LOC687308
RGD1309676 similar to RIKEN cDNA 5730469M10
RGD1309922 similar to 2610301G19Rik protein
RHOA1 ras homolog gene family, member A; ras homolog gene family, member C
RPL10A ribosomal protein L10A; similar to ribosomal protein L10a
RPS15A ribosomal protein S15a
SARS1 seryl-tRNA synthetase
SDHA1 succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
SFXN1 sideroflexin 1
SHMT1 serine hydroxymethyltransferase 1 (soluble)
SLC16A1 solute carrier family 16, member 1 (monocarboxylic acid transporter 1)
SLC25A1 solute carrier family 25 (mitochondrial carrier, citrate transporter), member 1
SLC25A11 solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11
SLC25A20 solute carrier family 25 (carnitine/acylcarnitine translocase), member 20
SLC25A3 solute carrier family 25 (mitochondrial carrier, phosphate carrier), member 3
SLC25A4 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 4
SLC25A5 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5
SNCB2 synuclein, beta
SND1 staphylococcal nuclease and tudor domain containing 1
SOD12 superoxide dismutase 1, soluble
STOML22 stomatin (Epb7.2)-like 2
STXBP1 syntaxin binding protein 1
SUCLA21 succinate-CoA ligase, ADP-forming, beta subunit
SURF4 surfeit 1; surfeit 4
SYNJ2BP synaptojanin 2 binding protein
TBC1D15 TBC1 domain family, member 15
TFAM2 transcription factor A, mitochondrial
TFRC transferrin receptor
TH tyrosine hydroxylase
TIMM92 translocase of inner mitochondrial membrane 9 homolog (yeast)
TOMM70A translocase of outer mitochondrial membrane 70 homolog A (S. cerevisiae)
TST thiosulfate sulfurtransferase, mitochondrial
TXN22 thioredoxin 2
UQCRFS12 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
VARS1 valyl-tRNA synthetase 2, mitochondrial (putative); valyl-tRNA synthetase
VDAC12 voltage-dependent anion channel 1
VDAC3 voltage-dependent anion channel 3
1

Proteins that also bind purines.

2

Proteins that were significantly increased; all of the rest were decreased.