mitochondrial matrix Gene Set

Dataset GO Cellular Component Annotations 2023
Category structural or functional annotations
Type biological process
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

356 proteins localized to the mitochondrial matrix cellular component from the curated GO Cellular Component Annotations 2023 dataset.

Symbol Name
AADAT aminoadipate aminotransferase
AASS aminoadipate-semialdehyde synthase
ABAT 4-aminobutyrate aminotransferase
ABCE1 ATP-binding cassette, sub-family E (OABP), member 1
ABHD10 abhydrolase domain containing 10
ACAA2 acetyl-CoA acyltransferase 2
ACAD10 acyl-CoA dehydrogenase family, member 10
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
ACOT2 acyl-CoA thioesterase 2
ACOT9 acyl-CoA thioesterase 9
ACP6 acid phosphatase 6, lysophosphatidic
ACSF2 acyl-CoA synthetase family member 2
ACSF3 acyl-CoA synthetase family member 3
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
ACSM6 acyl-CoA synthetase medium-chain family member 6
ACSS1 acyl-CoA synthetase short-chain family member 1
ACSS2 acyl-CoA synthetase short-chain family member 2
ACSS3 acyl-CoA synthetase short-chain family member 3
ADHFE1 alcohol dehydrogenase, iron containing, 1
ADPRS ADP-ribosylserine hydrolase
AGXT2 alanine--glyoxylate aminotransferase 2
AK4 adenylate kinase 4
AKR1B15 aldo-keto reductase family 1, member B15
ALAS1 5'-aminolevulinate synthase 1
ALAS2 5'-aminolevulinate synthase 2
ALDH1B1 aldehyde dehydrogenase 1 family, member B1
ALDH1L2 aldehyde dehydrogenase 1 family, member L2
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)
AMT aminomethyltransferase
ARG2 arginase 2
ARHGAP11B Rho GTPase activating protein 11B
ARL2 ADP-ribosylation factor-like 2
ARL2BP ADP-ribosylation factor-like 2 binding protein
ASPNAT aspartate N-acetyltransferase
ATP5F1A ATP synthase F1 subunit alpha
ATP5F1B ATP synthase F1 subunit beta
ATP5F1C ATP synthase F1 subunit gamma
ATP5F1D ATP synthase F1 subunit delta
ATP5F1E ATP synthase F1 subunit epsilon
ATP5PB ATP synthase peripheral stalk-membrane subunit b
ATXN3 ataxin 3
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
BCKDK branched chain ketoacid dehydrogenase kinase
BCO2 beta-carotene oxygenase 2
BDH1 3-hydroxybutyrate dehydrogenase, type 1
BLOC1S1 biogenesis of lysosomal organelles complex-1, subunit 1
BOLA3 bolA family member 3
BTD biotinidase
C1QBP complement component 1, q subcomponent binding protein
CA5A carbonic anhydrase VA, mitochondrial
CA5B carbonic anhydrase VB, mitochondrial
CASQ1 calsequestrin 1 (fast-twitch, skeletal muscle)
CBR4 carbonyl reductase 4
CCAR2 cell cycle and apoptosis regulator 2
CCNB1 cyclin B1
CDK1 cyclin-dependent kinase 1
CLPP caseinolytic mitochondrial matrix peptidase proteolytic subunit
CLPX caseinolytic mitochondrial matrix peptidase chaperone subunit
COASY CoA synthase
COQ3 coenzyme Q3 methyltransferase
COQ5 coenzyme Q5 homolog, methyltransferase (S. cerevisiae)
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
DGLUCY D-glutamate cyclase
DGUOK deoxyguanosine kinase
DHFRL1 dihydrofolate reductase-like 1
DHTKD1 dehydrogenase E1 and transketolase domain containing 1
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
DNAJC15 DnaJ (Hsp40) homolog, subfamily C, member 15
DNAJC19 DnaJ (Hsp40) homolog, subfamily C, member 19
ECHS1 enoyl CoA hydratase, short chain, 1, mitochondrial
ECI1 enoyl-CoA delta isomerase 1
ELAC2 elaC ribonuclease Z 2
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
ETNPPL ethanolamine-phosphate phospho-lyase
FAHD1 fumarylacetoacetate hydrolase domain containing 1
FARS2 phenylalanyl-tRNA synthetase 2, mitochondrial
FASTK Fas-activated serine/threonine kinase
FASTKD1 FAST kinase domains 1
FASTKD2 FAST kinase domains 2
FASTKD3 FAST kinase domains 3
FASTKD5 FAST kinase domains 5
FDX1 ferredoxin 1
FDX2 ferredoxin 2
FDXR ferredoxin reductase
FECH ferrochelatase
FH fumarate hydratase
FOXO3 forkhead box O3
FOXO3B forkhead box O3B pseudogene
FPGS folylpolyglutamate synthase
FTMT ferritin mitochondrial
FXN frataxin
GADD45GIP1 growth arrest and DNA-damage-inducible, gamma interacting protein 1
GARS1 glycyl-tRNA synthetase 1
GCDH glutaryl-CoA dehydrogenase
GCSH glycine cleavage system protein H (aminomethyl carrier)
GFM1 G elongation factor, mitochondrial 1
GFM2 G elongation factor, mitochondrial 2
GLDC glycine dehydrogenase (decarboxylating)
GLRX5 glutaredoxin 5
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
GSTZ1 glutathione S-transferase zeta 1
HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
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)
HOGA1 4-hydroxy-2-oxoglutarate aldolase 1
HSD17B10 hydroxysteroid (17-beta) dehydrogenase 10
HSD17B8 hydroxysteroid (17-beta) dehydrogenase 8
HSP90B2P heat shock protein 90kDa beta (Grp94), member 2, pseudogene
HSPA9 heat shock 70kDa protein 9 (mortalin)
HSPD1 heat shock 60kDa protein 1 (chaperonin)
HSPE1 heat shock 10kDa protein 1
HTD2 hydroxyacyl-thioester dehydratase type 2
HYKK hydroxylysine kinase
IARS2 isoleucyl-tRNA synthetase 2, mitochondrial
IBA57 IBA57, iron-sulfur cluster assembly homolog (S. cerevisiae)
IDH2 isocitrate dehydrogenase 2 (NADP+), mitochondrial
IDH3A isocitrate dehydrogenase 3 (NAD+) alpha
IDH3B isocitrate dehydrogenase 3 (NAD+) beta
IDH3G isocitrate dehydrogenase 3 (NAD+) gamma
ISCA1 iron-sulfur cluster assembly 1
ISCA2 iron-sulfur cluster assembly 2
ISCU iron-sulfur cluster assembly enzyme
IVD isovaleryl-CoA dehydrogenase
KARS1 lysyl-tRNA synthetase 1
KRT17 keratin 17, type I
LACTB2 lactamase, beta 2
LARS2 leucyl-tRNA synthetase 2, mitochondrial
LDHAL6B lactate dehydrogenase A-like 6B
LIAS lipoic acid synthetase
LIPT1 lipoyltransferase 1
LIPT2 lipoyl(octanoyl) transferase 2 (putative)
LONP1 lon peptidase 1, mitochondrial
LRRK2 leucine-rich repeat kinase 2
LYRM4 LYR motif containing 4
LYRM7 LYR motif containing 7
MAIP1 matrix AAA peptidase interacting protein 1
MARS2 methionyl-tRNA synthetase 2, mitochondrial
MCAT malonyl CoA:ACP acyltransferase (mitochondrial)
MCCC1 methylcrotonoyl-CoA carboxylase 1 (alpha)
MCCC2 methylcrotonoyl-CoA carboxylase 2 (beta)
MCEE methylmalonyl CoA epimerase
MDH2 malate dehydrogenase 2, NAD (mitochondrial)
ME2 malic enzyme 2, NAD(+)-dependent, mitochondrial
ME3 malic enzyme 3, NADP(+)-dependent, mitochondrial
MECR mitochondrial trans-2-enoyl-CoA reductase
METTL15 methyltransferase like 15
METTL17 methyltransferase like 17
METTL20 methyltransferase like 20
METTL4 methyltransferase like 4
MLYCD malonyl-CoA decarboxylase
MMAA methylmalonic aciduria (cobalamin deficiency) cblA type
MMAB methylmalonic aciduria (cobalamin deficiency) cblB type
MMUT methylmalonyl-CoA mutase
MPST mercaptopyruvate sulfurtransferase
MRM1 mitochondrial rRNA methyltransferase 1 homolog (S. cerevisiae)
MRM2 mitochondrial rRNA methyltransferase 2
MRM3 mitochondrial rRNA methyltransferase 3
MRPL11 mitochondrial ribosomal protein L11
MRPL13 mitochondrial ribosomal protein L13
MRPL14 mitochondrial ribosomal protein L14
MRPL18 mitochondrial ribosomal protein L18
MRPL20 mitochondrial ribosomal protein L20
MRPL28 mitochondrial ribosomal protein L28
MRPL34 mitochondrial ribosomal protein L34
MRPL35 mitochondrial ribosomal protein L35
MRPL37 mitochondrial ribosomal protein L37
MRPL39 mitochondrial ribosomal protein L39
MRPL40 mitochondrial ribosomal protein L40
MRPL43 mitochondrial ribosomal protein L43
MRPL48 mitochondrial ribosomal protein L48
MRPL49 mitochondrial ribosomal protein L49
MRPL51 mitochondrial ribosomal protein L51
MRPL57 mitochondrial ribosomal protein L57
MRPL58 mitochondrial ribosomal protein L58
MRPL9 mitochondrial ribosomal protein L9
MRPS12 mitochondrial ribosomal protein S12
MRPS14 mitochondrial ribosomal protein S14
MRPS22 mitochondrial ribosomal protein S22
MRRF mitochondrial ribosome recycling factor
MTERF1 mitochondrial transcription termination factor 1
MTERF2 mitochondrial transcription termination factor 2
MTERF4 mitochondrial transcription termination factor 4
MTG1 mitochondrial ribosome-associated GTPase 1
MTG2 mitochondrial ribosome-associated GTPase 2
MTHFD1L methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like
MTHFD2 methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase
MTHFD2L methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2-like
MTHFS 5,10-methenyltetrahydrofolate synthetase (5-formyltetrahydrofolate cyclo-ligase)
MTRES1 mitochondrial transcription rescue factor 1
MTRF1L mitochondrial translational release factor 1-like
NADK2 NAD kinase 2, mitochondrial
NAGS N-acetylglutamate synthase
NAXD NAD(P)HX dehydratase
NAXE NAD(P)HX epimerase
NDUFA7 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa
NDUFA9 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
NDUFAB1 NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa
NDUFAF7 NADH dehydrogenase (ubiquinone) complex I, assembly factor 7
NDUFB8 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa
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)
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)
NFS1 NFS1 cysteine desulfurase
NMNAT3 nicotinamide nucleotide adenylyltransferase 3
NR3C1 nuclear receptor subfamily 3, group C, member 1 (glucocorticoid receptor)
NSUN3 NOP2/Sun domain family, member 3
NSUN4 NOP2/Sun domain family, member 4
NT5M 5',3'-nucleotidase, mitochondrial
NUBPL nucleotide binding protein-like
NUDT1 nudix (nucleoside diphosphate linked moiety X)-type motif 1
NUDT13 nudix (nucleoside diphosphate linked moiety X)-type motif 13
NUDT2 nudix (nucleoside diphosphate linked moiety X)-type motif 2
NUDT9 nudix (nucleoside diphosphate linked moiety X)-type motif 9
OAT ornithine aminotransferase
OGDH oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
OGDHL oxoglutarate dehydrogenase-like
OGG1 8-oxoguanine DNA glycosylase
OTC ornithine carbamoyltransferase
OXA1L oxidase (cytochrome c) assembly 1-like
OXCT1 3-oxoacid CoA transferase 1
OXCT2 3-oxoacid CoA transferase 2
PAM16 presequence translocase-associated motor 16 homolog (S. cerevisiae)
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
PHYKPL 5-phosphohydroxy-L-lysine phospho-lyase
PITRM1 pitrilysin metallopeptidase 1
POLDIP2 polymerase (DNA-directed), delta interacting protein 2
POLG polymerase (DNA directed), gamma
POLG2 polymerase (DNA directed), gamma 2, accessory subunit
POLRMT polymerase (RNA) mitochondrial (DNA directed)
PPA2 pyrophosphatase (inorganic) 2
PPM1K protein phosphatase, Mg2+/Mn2+ dependent, 1K
PPTC7 PTC7 protein phosphatase homolog (S. cerevisiae)
PRDX3 peroxiredoxin 3
PRDX5 peroxiredoxin 5
PRIMPOL primase and polymerase (DNA-directed)
PRORP protein only RNase P catalytic subunit
PTCD1 pentatricopeptide repeat domain 1
PUS1 pseudouridylate synthase 1
PYCR1 pyrroline-5-carboxylate reductase 1
PYCR2 pyrroline-5-carboxylate reductase family, member 2
PYROXD2 pyridine nucleotide-disulphide oxidoreductase domain 2
QARS1 glutaminyl-tRNA synthetase 1
RARS2 arginyl-tRNA synthetase 2, mitochondrial
REXO2 RNA exonuclease 2
RIDA reactive intermediate imine deaminase A homolog
RNASEL ribonuclease L (2',5'-oligoisoadenylate synthetase-dependent)
RPS3 ribosomal protein S3
SARDH sarcosine dehydrogenase
SARS2 seryl-tRNA synthetase 2, mitochondrial
SCO2 SCO2 cytochrome c oxidase assembly protein
SHMT2 serine hydroxymethyltransferase 2 (mitochondrial)
SIRT3 sirtuin 3
SIRT4 sirtuin 4
SIRT5 sirtuin 5
SMDT1 single-pass membrane protein with aspartate-rich tail 1
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)
TBRG4 transforming growth factor beta regulator 4
TEFM transcription elongation factor, mitochondrial
TFAM transcription factor A, mitochondrial
TFB1M transcription factor B1, mitochondrial
TFB2M transcription factor B2, mitochondrial
THEM4 thioesterase superfamily member 4
THEM5 thioesterase superfamily member 5
TIMM44 translocase of inner mitochondrial membrane 44 homolog (yeast)
TK1 thymidine kinase 1, soluble
TMLHE trimethyllysine hydroxylase, epsilon
TP53AIP1 tumor protein p53 regulated apoptosis inducing protein 1
TRIT1 tRNA isopentenyltransferase 1
TRMT10C tRNA methyltransferase 10 homolog C (S. cerevisiae)
TRMT5 tRNA methyltransferase 5
TRMT61B tRNA methyltransferase 61B
TRNT1 tRNA nucleotidyl transferase, CCA-adding, 1
TSFM Ts translation elongation factor, mitochondrial
TST thiosulfate sulfurtransferase (rhodanese)
TWNK twinkle mtDNA helicase
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