mitochondrial matrix Gene Set

Dataset GO Cellular Component Annotations
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)
External Link http://amigo.geneontology.org/amigo/term/GO:0005759
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Genes

263 proteins localized to the mitochondrial matrix cellular component from the curated GO Cellular Component Annotations dataset.

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