mitochondrial matrix Gene Set

Dataset COMPARTMENTS Curated Protein Localization Evidence Scores
Category structural or functional annotations
Type cellular component
Description The gel-like material, with considerable fine structure, that lies in the matrix space, or lumen, of a mitochondrion. It contains the enzymes of the tricarboxylic acid cycle and, in some organisms, the enzymes concerned with fatty acid oxidation. (Gene Ontology, GO_0005759)
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Genes

294 proteins localized to the mitochondrial matrix cellular component from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.

Symbol Name Standardized Value
IVD isovaleryl-CoA dehydrogenase 0.663342
DHRS2 dehydrogenase/reductase (SDR family) member 2 0.663342
HSPE1 heat shock 10kDa protein 1 0.663342
ACADM acyl-CoA dehydrogenase, C-4 to C-12 straight chain 0.663342
ACADL acyl-CoA dehydrogenase, long chain 0.663342
ACADS acyl-CoA dehydrogenase, C-2 to C-3 short chain 0.663342
DBT dihydrolipoamide branched chain transacylase E2 0.663342
WARS2 tryptophanyl tRNA synthetase 2, mitochondrial 0.663342
CBR4 carbonyl reductase 4 0.663342
GRSF1 G-rich RNA sequence binding factor 1 0.663342
ECHS1 enoyl CoA hydratase, short chain, 1, mitochondrial 0.663342
TP53 tumor protein p53 0.663342
PITRM1 pitrilysin metallopeptidase 1 0.663342
GRPEL1 GrpE-like 1, mitochondrial (E. coli) 0.663342
MRPL28 mitochondrial ribosomal protein L28 0.663342
MRPL20 mitochondrial ribosomal protein L20 0.663342
MRPL27 mitochondrial ribosomal protein L27 0.663342
DNAJA3 DnaJ (Hsp40) homolog, subfamily A, member 3 0.663342
ATG4D autophagy related 4D, cysteine peptidase 0.663342
RAD51 RAD51 recombinase 0.663342
ATAD3A ATPase family, AAA domain containing 3A 0.663342
DHX30 DEAH (Asp-Glu-Ala-His) box helicase 30 0.663342
THEM5 thioesterase superfamily member 5 0.663342
ALDH2 aldehyde dehydrogenase 2 family (mitochondrial) 0.663342
MUT methylmalonyl CoA mutase 0.663342
CLPX caseinolytic mitochondrial matrix peptidase chaperone subunit 0.663342
ATP5B ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide 0.663342
ME2 malic enzyme 2, NAD(+)-dependent, mitochondrial 0.663342
ME3 malic enzyme 3, NADP(+)-dependent, mitochondrial 0.663342
MRPS21 mitochondrial ribosomal protein S21 0.663342
MRPS22 mitochondrial ribosomal protein S22 0.663342
MRPS24 mitochondrial ribosomal protein S24 0.663342
MRPS26 mitochondrial ribosomal protein S26 0.663342
MRPS28 mitochondrial ribosomal protein S28 0.663342
CLPP caseinolytic mitochondrial matrix peptidase proteolytic subunit 0.663342
ACSM1 acyl-CoA synthetase medium-chain family member 1 0.663342
MTERF4 mitochondrial transcription termination factor 4 0.663342
ERAL1 Era-like 12S mitochondrial rRNA chaperone 1 0.663342
TFAM transcription factor A, mitochondrial 0.663342
PIN4 peptidylprolyl cis/trans isomerase, NIMA-interacting 4 0.663342
SHMT2 serine hydroxymethyltransferase 2 (mitochondrial) 0.663342
TST thiosulfate sulfurtransferase (rhodanese) 0.663342
PARG poly (ADP-ribose) glycohydrolase 0.663342
MTG1 mitochondrial ribosome-associated GTPase 1 0.663342
MTG2 mitochondrial ribosome-associated GTPase 2 0.663342
BCKDK branched chain ketoacid dehydrogenase kinase 0.663342
MRPL48 mitochondrial ribosomal protein L48 0.663342
FLAD1 flavin adenine dinucleotide synthetase 1 0.663342
MRPL36 mitochondrial ribosomal protein L36 0.663342
ATP5F1 ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1 0.663342
OAT ornithine aminotransferase 0.663342
ECI1 enoyl-CoA delta isomerase 1 0.663342
UQCC2 ubiquinol-cytochrome c reductase complex assembly factor 2 0.663342
MRPL9 mitochondrial ribosomal protein L9 0.663342
MRPL3 mitochondrial ribosomal protein L3 0.663342
FPGS folylpolyglutamate synthase 0.663342
NDUFA10 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa 0.663342
MRPL37 mitochondrial ribosomal protein L37 0.663342
MRPL34 mitochondrial ribosomal protein L34 0.663342
MRPL32 mitochondrial ribosomal protein L32 0.663342
ADPRHL2 ADP-ribosylhydrolase like 2 0.663342
MIPEP mitochondrial intermediate peptidase 0.663342
MRPS33 mitochondrial ribosomal protein S33 0.663342
MRPS36 mitochondrial ribosomal protein S36 0.663342
HADH hydroxyacyl-CoA dehydrogenase 0.663342
COASY CoA synthase 0.663342
CS citrate synthase 0.663342
BCKDHA branched chain keto acid dehydrogenase E1, alpha polypeptide 0.663342
BCKDHB branched chain keto acid dehydrogenase E1, beta polypeptide 0.663342
REXO2 RNA exonuclease 2 0.663342
TRAP1 TNF receptor-associated protein 1 0.663342
ICT1 immature colon carcinoma transcript 1 0.663342
ZNF713 zinc finger protein 713 0.663342
DLAT dihydrolipoamide S-acetyltransferase 0.663342
NAGS N-acetylglutamate synthase 0.663342
SIRT4 sirtuin 4 0.663342
SIRT5 sirtuin 5 0.663342
NDUFA9 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa 0.663342
PRODH proline dehydrogenase (oxidase) 1 0.663342
ETFB electron-transfer-flavoprotein, beta polypeptide 0.663342
ETFA electron-transfer-flavoprotein, alpha polypeptide 0.663342
DLD dihydrolipoamide dehydrogenase 0.663342
NUDT1 nudix (nucleoside diphosphate linked moiety X)-type motif 1 0.663342
FDXR ferredoxin reductase 0.663342
MRPL35 mitochondrial ribosomal protein L35 0.663342
ALDH4A1 aldehyde dehydrogenase 4 family, member A1 0.663342
MRPL41 mitochondrial ribosomal protein L41 0.663342
DMGDH dimethylglycine dehydrogenase 0.663342
GCDH glutaryl-CoA dehydrogenase 0.663342
HMGCL 3-hydroxymethyl-3-methylglutaryl-CoA lyase 0.663342
GLUD1 glutamate dehydrogenase 1 0.663342
MDH2 malate dehydrogenase 2, NAD (mitochondrial) 0.663342
MRPL51 mitochondrial ribosomal protein L51 0.663342
OXCT1 3-oxoacid CoA transferase 1 0.663342
PDK4 pyruvate dehydrogenase kinase, isozyme 4 0.663342
ACADSB acyl-CoA dehydrogenase, short/branched chain 0.663342
MRPS16 mitochondrial ribosomal protein S16 0.663342
MRPS15 mitochondrial ribosomal protein S15 0.663342
MRPS14 mitochondrial ribosomal protein S14 0.663342
ETHE1 ethylmalonic encephalopathy 1 0.663342
TEFM transcription elongation factor, mitochondrial 0.663342
ACSS1 acyl-CoA synthetase short-chain family member 1 0.663342
PRIMPOL primase and polymerase (DNA-directed) 0.663342
MARS2 methionyl-tRNA synthetase 2, mitochondrial 0.663342
PDK2 pyruvate dehydrogenase kinase, isozyme 2 0.663342
PDK3 pyruvate dehydrogenase kinase, isozyme 3 0.663342
PDHA2 pyruvate dehydrogenase (lipoamide) alpha 2 0.663342
PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 0.663342
HSD17B8 hydroxysteroid (17-beta) dehydrogenase 8 0.663342
PC pyruvate carboxylase 0.663342
C1QBP complement component 1, q subcomponent binding protein 0.663342
SUPV3L1 suppressor of var1, 3-like 1 (S. cerevisiae) 0.663342
PDHB pyruvate dehydrogenase (lipoamide) beta 0.663342
HAGH hydroxyacylglutathione hydrolase 0.663342
MRPL11 mitochondrial ribosomal protein L11 0.663342
MCCC1 methylcrotonoyl-CoA carboxylase 1 (alpha) 0.663342
MRPL12 mitochondrial ribosomal protein L12 0.663342
MRPL13 mitochondrial ribosomal protein L13 0.663342
MRPL18 mitochondrial ribosomal protein L18 0.663342
PDHX pyruvate dehydrogenase complex, component X 0.663342
AGXT alanine-glyoxylate aminotransferase 0.663342
ALKBH7 alkB, alkylation repair homolog 7 (E. coli) 0.663342
SOD2 superoxide dismutase 2, mitochondrial 0.663342
SOD1 superoxide dismutase 1, soluble 0.663342
CCAR2 cell cycle and apoptosis regulator 2 0.663342
MRPS18A mitochondrial ribosomal protein S18A 0.663342
DHFRL1 dihydrofolate reductase-like 1 0.663342
DNA2 DNA replication helicase/nuclease 2 0.663342
MRPL43 mitochondrial ribosomal protein L43 0.663342
OTC ornithine carbamoyltransferase 0.663342
HSPD1 heat shock 60kDa protein 1 (chaperonin) 0.663342
TFB2M transcription factor B2, mitochondrial 0.663342
ALDH1B1 aldehyde dehydrogenase 1 family, member B1 0.663342
ALAS1 5'-aminolevulinate synthase 1 0.663342
MRPL39 mitochondrial ribosomal protein L39 0.663342
BDH1 3-hydroxybutyrate dehydrogenase, type 1 0.663342
OGDH oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) 0.663342
TYMS thymidylate synthetase 0.663342
MRPL42 mitochondrial ribosomal protein L42 0.663342
MRPS9 mitochondrial ribosomal protein S9 0.663342
BLOC1S1 biogenesis of lysosomal organelles complex-1, subunit 1 0.663342
MRPS17 mitochondrial ribosomal protein S17 0.663342
C10ORF2 chromosome 10 open reading frame 2 0.663342
NSUN4 NOP2/Sun domain family, member 4 0.663342
MRPS18B mitochondrial ribosomal protein S18B 0.663342
MRPS18C mitochondrial ribosomal protein S18C 0.663342
MRPL49 mitochondrial ribosomal protein L49 0.663342
ABAT 4-aminobutyrate aminotransferase 0.663342
BTD biotinidase 0.237908
MMAA methylmalonic aciduria (cobalamin deficiency) cblA type 0.237908
GLS glutaminase 0.237908
FECH ferrochelatase 0.237908
MMAB methylmalonic aciduria (cobalamin deficiency) cblB type 0.237908
CA5B carbonic anhydrase VB, mitochondrial 0.237908
ADHFE1 alcohol dehydrogenase, iron containing, 1 0.237908
HIBCH 3-hydroxyisobutyryl-CoA hydrolase 0.237908
TUFM Tu translation elongation factor, mitochondrial 0.237908
LYRM4 LYR motif containing 4 0.237908
TOP1MT topoisomerase (DNA) I, mitochondrial 0.237908
MLYCD malonyl-CoA decarboxylase 0.237908
GLS2 glutaminase 2 (liver, mitochondrial) 0.237908
ERBB4 erb-b2 receptor tyrosine kinase 4 0.237908
PYCR1 pyrroline-5-carboxylate reductase 1 0.237908
PPA2 pyrophosphatase (inorganic) 2 0.237908
QARS glutaminyl-tRNA synthetase 0.237908
SUCLA2 succinate-CoA ligase, ADP-forming, beta subunit 0.237908
IDH2 isocitrate dehydrogenase 2 (NADP+), mitochondrial 0.237908
IDH3A isocitrate dehydrogenase 3 (NAD+) alpha 0.237908
AASS aminoadipate-semialdehyde synthase 0.237908
ALDH7A1 aldehyde dehydrogenase 7 family, member A1 0.237908
DGUOK deoxyguanosine kinase 0.237908
HSPA9 heat shock 70kDa protein 9 (mortalin) 0.237908
DLST dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex) 0.237908
SLC25A5 solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 5 0.237908
IARS2 isoleucyl-tRNA synthetase 2, mitochondrial 0.237908
VDAC2 voltage-dependent anion channel 2 0.237908
VDAC1 voltage-dependent anion channel 1 0.237908
DECR1 2,4-dienoyl CoA reductase 1, mitochondrial 0.237908
PDK1 pyruvate dehydrogenase kinase, isozyme 1 0.237908
SUOX sulfite oxidase 0.237908
GOT2 glutamic-oxaloacetic transaminase 2, mitochondrial 0.237908
TMLHE trimethyllysine hydroxylase, epsilon 0.237908
TIMM44 translocase of inner mitochondrial membrane 44 homolog (yeast) 0.237908
POLG2 polymerase (DNA directed), gamma 2, accessory subunit 0.237908
ETFDH electron-transferring-flavoprotein dehydrogenase 0.237908
DARS2 aspartyl-tRNA synthetase 2, mitochondrial 0.237908
SUCLG2 succinate-CoA ligase, GDP-forming, beta subunit 0.237908
SUCLG1 succinate-CoA ligase, alpha subunit 0.237908
FH fumarate hydratase 0.237908
MRPS35 mitochondrial ribosomal protein S35 0.237908
CYP11A1 cytochrome P450, family 11, subfamily A, polypeptide 1 0.237908
SSBP1 single-stranded DNA binding protein 1, mitochondrial 0.237908
CASQ1 calsequestrin 1 (fast-twitch, skeletal muscle) 0.237908
RARS2 arginyl-tRNA synthetase 2, mitochondrial 0.237908
NDUFAB1 NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa 0.237908
YARS2 tyrosyl-tRNA synthetase 2, mitochondrial 0.237908
GSR glutathione reductase 0.237908
PCK2 phosphoenolpyruvate carboxykinase 2 (mitochondrial) 0.237908
PCCB propionyl CoA carboxylase, beta polypeptide 0.237908
FBXO17 F-box protein 17 0.237908
NR3C1 nuclear receptor subfamily 3, group C, member 1 (glucocorticoid receptor) 0.237908
PDSS1 prenyl (decaprenyl) diphosphate synthase, subunit 1 0.237908
PDSS2 prenyl (decaprenyl) diphosphate synthase, subunit 2 0.237908
FDX1L ferredoxin 1-like 0.237908
TXNRD2 thioredoxin reductase 2 0.237908
ATP5C1 ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 0.237908
PCCA propionyl CoA carboxylase, alpha polypeptide 0.237908
GPX1 glutathione peroxidase 1 0.237908
CPS1 carbamoyl-phosphate synthase 1, mitochondrial 0.237908
MCEE methylmalonyl CoA epimerase 0.237908
MRPL40 mitochondrial ribosomal protein L40 0.237908
ARG2 arginase 2 0.237908
POLG polymerase (DNA directed), gamma 0.237908
PRDX5 peroxiredoxin 5 0.237908
PRDX3 peroxiredoxin 3 0.237908
MRPS12 mitochondrial ribosomal protein S12 0.237908
ACAD8 acyl-CoA dehydrogenase family, member 8 0.237908
FARS2 phenylalanyl-tRNA synthetase 2, mitochondrial 0.237908
AGXT2 alanine--glyoxylate aminotransferase 2 0.237908
IDH3B isocitrate dehydrogenase 3 (NAD+) beta 0.237908
IDH3G isocitrate dehydrogenase 3 (NAD+) gamma 0.237908
AUH AU RNA binding protein/enoyl-CoA hydratase 0.237908
GPT2 glutamic pyruvate transaminase (alanine aminotransferase) 2 0.237908
MRPS11 mitochondrial ribosomal protein S11 0.237908
MRPL23 mitochondrial ribosomal protein L23 0.237908
POLRMT polymerase (RNA) mitochondrial (DNA directed) 0.237908
MRPL10 mitochondrial ribosomal protein L10 0.237908
ACSM2B acyl-CoA synthetase medium-chain family member 2B 0.237908
HSD17B10 hydroxysteroid (17-beta) dehydrogenase 10 0.237908
ALAS2 5'-aminolevulinate synthase 2 0.237908
NFS1 NFS1 cysteine desulfurase 0.237908
ATP5E ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit 0.237908
ATP5D ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit 0.237908
ATP5A1 ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle 0.237908
KARS lysyl-tRNA synthetase 0.237908
PDE2A phosphodiesterase 2A, cGMP-stimulated 0.237908
ALDH6A1 aldehyde dehydrogenase 6 family, member A1 0.237908
PDP2 pyruvate dehyrogenase phosphatase catalytic subunit 2 0.237908
ACO2 aconitase 2, mitochondrial 0.237908
HARS2 histidyl-tRNA synthetase 2, mitochondrial 0.237908
LONP1 lon peptidase 1, mitochondrial 0.237908
TK2 thymidine kinase 2, mitochondrial 0.237908
HMGCS2 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) 0.237908
ACADVL acyl-CoA dehydrogenase, very long chain 0.237908
GARS glycyl-tRNA synthetase 0.237908
BCAT2 branched chain amino-acid transaminase 2, mitochondrial 0.237908
PTCD1 pentatricopeptide repeat domain 1 0.237908
MRPL55 mitochondrial ribosomal protein L55 0.237908
MTERF2 mitochondrial transcription termination factor 2 0.237908
MTERF1 mitochondrial transcription termination factor 1 0.237908
PDPR pyruvate dehydrogenase phosphatase regulatory subunit 0.237908
LARS2 leucyl-tRNA synthetase 2, mitochondrial 0.237908
ALDH5A1 aldehyde dehydrogenase 5 family, member A1 0.237908
MRPL52 mitochondrial ribosomal protein L52 0.237908
LRRC59 leucine rich repeat containing 59 0.237908
HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit 0.237908
TXN2 thioredoxin 2 0.237908
LRPPRC leucine-rich pentatricopeptide repeat containing 0.237908
HADHB hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit 0.237908
PDP1 pyruvate dehyrogenase phosphatase catalytic subunit 1 0.237908
D2HGDH D-2-hydroxyglutarate dehydrogenase 0.237908
FDX1 ferredoxin 1 0.237908
ACAT1 acetyl-CoA acetyltransferase 1 0.237908
DDX28 DEAD (Asp-Glu-Ala-Asp) box polypeptide 28 0.237908
CA5A carbonic anhydrase VA, mitochondrial 0.237908
MCCC2 methylcrotonoyl-CoA carboxylase 2 (beta) 0.237908
NT5M 5',3'-nucleotidase, mitochondrial 0.237908
TFB1M transcription factor B1, mitochondrial 0.237908
COQ3 coenzyme Q3 methyltransferase 0.237908
LRRK2 leucine-rich repeat kinase 2 0.237908
ISCU iron-sulfur cluster assembly enzyme 0.237908
AADAT aminoadipate aminotransferase 0.237908
CYP27A1 cytochrome P450, family 27, subfamily A, polypeptide 1 0.237908
GLYAT glycine-N-acyltransferase 0.237908
HIBADH 3-hydroxyisobutyrate dehydrogenase 0.237908
NUDT9 nudix (nucleoside diphosphate linked moiety X)-type motif 9 0.237908
AK3 adenylate kinase 3 0.098109
FXN frataxin 0.098109
MPG N-methylpurine-DNA glycosylase 0.098109
TRMT10C tRNA methyltransferase 10 homolog C (S. cerevisiae) 0.098109
DAP3 death associated protein 3 0.03318
MRPL47 mitochondrial ribosomal protein L47 0.03318
DTYMK deoxythymidylate kinase (thymidylate kinase) 0.03318
TDRD7 tudor domain containing 7 0.03318
MCL1 myeloid cell leukemia 1 0.03318
TRMT5 tRNA methyltransferase 5 0.03318
MRPL57 mitochondrial ribosomal protein L57 0.03318
GSTK1 glutathione S-transferase kappa 1 0.03318
PRDX1 peroxiredoxin 1 0.03318
ACSM3 acyl-CoA synthetase medium-chain family member 3 0.03318
PPIF peptidylprolyl isomerase F 0.03318
MRPS31 mitochondrial ribosomal protein S31 0.03318
GUF1 GUF1 GTPase homolog (S. cerevisiae) 0.03318
HSPA1L heat shock 70kDa protein 1-like 0.03318