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MetaCyc Gene-Ontology-Terms Class: GO:0005739 - mitochondrion

Synonyms: mitochondria

Definition: A semiautonomous, self replicating organelle that occurs in varying numbers, shapes, and sizes in the cytoplasm of virtually all eukaryotic cells. It is notably the site of tissue respiration.

Some anaerobic or microaerophilic organisms (e.g. Entamoeba histolytica, Giardia intestinalis and several Microsporidia species) do not have mitochondria, and contain mitochondrion-related organelles (MROs) instead, called mitosomes or hydrogenosomes, very likely derived from mitochondria. To annotate gene products located in these mitochondrial relics in species such as Entamoeba histolytica, Giardia intestinalis or others, please use GO:0032047 'mitosome' or GO:0042566 'hydrogenosome'. (See PMID:24316280 for a list of species currently known to contain mitochondrion-related organelles.)

Parent Classes:
GO:0043231 - intracellular membrane-bounded organelle,
GO:0044444 - cytoplasmic part

Part Of:
GO:0005737 - cytoplasm

GO:0098798 - mitochondrial protein complex (2),
GO:0044429 - mitochondrial part (140),
GO:0005759 - mitochondrial matrix (65),
GO:0005740 - mitochondrial envelope (1)

Term Members:
3-hydroxypropanonate dehydrogenase (HPD1),
3-hydroxypropanonyl-CoA hydrolase (EHD3),
Superoxide dismutase [Mn], mitochondrial (SOD2),
ubiquinone biosynthesis protein COQ7 homolog,
hexaprenyldihydroxybenzoate methyltransferase (COQ3),
inactive L-threonine 3-dehydrogenase (TDH),
2-amino-3-ketobutyrate coenzyme A ligase (GCAT),
carnitine O-palmitoyltransferase 2 (CPT2),
mitochondrial sulfite oxidase (SUOX),
carnitine O-acetyltransferase (CRAT),
mitochondrial carnitine/acylcarnitine carrier protein (SLC25A20),
NAD(P) transhydrogenase, mitochondrial (NNT),
Aspartate aminotransferase, mitochondrial (GOT2),
Methylmalonyl-CoA mutase, mitochondrial,
Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB),
Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA),
succinyl-CoA synthetase,
Arginase-2, mitochondrial (ARG2),
succinyl-CoA:acetate CoA-transferase (ASCT),
Glycine amidinotransferase (GATM),
Succinate-semialdehyde dehydrogenase, mitochondrial (ALDH5A1),
dimethylglycine dehydrogenase (DMGDH),
Ornithine carbamoyltransferase, mitochondrial (OTC),
δ-1-pyrroline-5-carboxylate synthase (ALDH18A1),
Glutamate dehydrogenase 1, mitochondrial (GLUD1),
Glutamate dehydrogenase 2, mitochondrial (GLUD2),
2-enoyl thioester reductase,
hydroxyacyl-thioester dehydratase (HTD2),
Trimethyllysine dioxygenase, mitochondrial (TMLHE),
folylpolyglutamate synthase (FPGS),
cytochrome P450 314a1, mitochondrial (shd),
Ferrochelatase, mitochondrial (FECH),
Protoporphyrinogen oxidase (PPOX),
Coproporphyrinogen-III oxidase, mitochondrial (CPOX),
5-aminolevulinate synthase, nonspecific, mitochondrial (ALAS1),
5-aminolevulinate synthase, erythroid-specific, mitochondrial (ALAS2),
cytochrome P450 315a1, mitochondrial (sad),
aminomethyltransferase (AMT),
glycine dehydrogenase [decarboxylating], mitochondrial (GLDC),
methionine synthase reductase (MTRR),
cytochrome P450 302a1, mitochondrial (dib),
alanine--glyoxylate aminotransferase 2, mitochondrial (AGXT2),
4-hydroxy-2-ketoglutarate aldolase,
4-hydroxy-2-oxoglutarate aldolase,
L-cysteine desulfurase (NFS1),
phosphoenolpyruvate carboxykinase [GTP], mitochondrial (PCK2),
pyruvate carboxylase, mitochondrial (PC),
Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial (IDH3A),
Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial (IDH3B),
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial (IDH3G),
mitochondrial deoxyguanosine kinase monomer (DGUOK),
thymidine kinase 2 (TK2),
mitochondrial deoxyuridine 5'-triphosphate nucleotidohydrolase monomer (DUT),
1,25-dihydroxyvitamin D(3) 24-hydroxylase, mitochondrial (CYP24A1),
long chain 3-hydroxyacyl-CoA dehydrogenase (HADHA),
mitochondrial enoyl-CoA hydratase (ECHS1),
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HADH),
trifunctional enzyme beta subunit, mitochondrial precursor (HADHB),
α-methylacyl-CoA racemase (AMACR),
pyruvate formate lyase (PFL1),
steroid 11β-hydroxylase (CYP11B1),
serine hydroxymethyltransferase (SHMT2),
malic enzyme (NAD),
malic enzyme (NAD),
aspartate transaminase (AAT3),
2-oxoglutarate decarboxylase,
glycine decarboxylase,
mitochondrial aldehyde dehydrogenase,
glycine decarboxylase,
benzaldehyde dehydrogenase,
mitochondrial aspartate aminotransferase,
glutaminase, liver isoform,
glycine cleavage complex,
glycine decarboxylase,
kynurenine/alpha-aminoadipate aminotransferase,
α-aminoadipate aminotransferase,
saccharopine dehydrogenase / lysine-ketoglutarate reductase,
β-cyano-L-alanine synthase,
propionyl-CoA carboxylase,
L-arginine:glycine amidinotransferase,
glutamate dehydrogenase (NAD-dependent),
glutamate dehydrogenase (NAD-dependent),
(-)-carveol dehydrogenase / (-)-isopiperitenol dehydrogenase,
malonate semialdehyde dehydrogenase,
β-alanine aminotransferase,
phosphatidylglycerolphosphate synthase,
O-acetylserine (thiol) lyase,
serine acetyltransferase,
β-alanine aminotransferase,
5-formyltetrahydrofolate cycloligase (LeFCL),
methylmalonyl-CoA mutase,
serine hydroxymethyltransferase,
glutathione reductase,
ascorbate peroxidase,
ornithine carbamoyltransferase,
malate dehydrogenase,
mitochondrial trans-2-enoyl-CoA reductase (TER),
citrate synthase (mitochondrial) (CS),
glutamine N-phenylacetyltransferase,
long-chain-fatty-acid-CoA ligase 1 (Acs1),
carnitine O-acetyltransferase (Crat),
lipoamide dehydrogenase (DLD),
aminomethyltransferase (AMT),
phosphatidylglycerolphosphate phosphatase,
phosphatidylglycerolphosphate synthase,
protoporphyrinogen IX oxidase isozyme II (PPXII),
Succinate dehydrogenase [ubiquinone] flavoprotein subunit (SDHA),
1-pyrroline-5-carboxylate dehydrogenase (At5g62530),
ornithine-δ-aminotransferase (δ-OAT),
dihydrofolate synthetase (DHFS),
proline dehydrogenase 2 (POX2),
NADH-ubiquinone oxidoreductase 75 kDa subunit (at5g37510),
glutamine synthetase, type II (GLN2),
5-formyltetrahydrofolate cycloligase (AtFCL),
cardiolipin synthase (AT4G04870),
ketopantoate hydroxymethyltransferase (PANB2),
β-cyano-L-alanine synthase (At3g61440),
proline dehydrogenase 1 (POX1),
adenylate isopentenyltransferase (IPT7),
γ-aminobutyrate transaminase (pyruvate dependent) (POP2),
superoxide dismutase (Mn) (MSD1),
folylpolyglutamate synthetase (DHFS/FPGS3),
ketopantoate hydroxymethyltransferase (PANB1),
citrate synthase (mitochondrial) (AT2G44350),
aminodeoxychorismate synthase (ADCS),
ornithine/ornithine exchanger / arginine/citrulline exchanger (at1g79900),
geranylgeranyl pyrophosphate synthase (GGPS6),
very-long-chain acyl-CoA dehydrogenase monomer (ACADVL),
long-chain-acyl-CoA dehydrogenase monomer (ACADL),
short-chain acyl-CoA dehydrogenase monomer (ACADS),
mitochondrial medium-chain acyl-CoA dehydrogenase monomer (ACADM),
pyruvate dehydrogenase E1 component β subunit (PDHB),
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial (PDHA2),
pyruvate dehydrogenase protein E3 component, mitochondrial (PDHX),
pyruvate dehydrogenase E2 component (DLAT),
pyruvate dehydrogenase E1 component α subunit (somatic) (PDHA1),
3-methylcrotonyl-CoA carboxylase β subunit (MCCC2),
mitochondrial glutaminase precursor (kidney isoform) (GLS),
3-ketoacyl-CoA thiolase, mitochondrial (ACAA2),
ubiquitous mitochondrial creatine kinase subunit (CKMT1B),
cholesterol side chain cleavage enzyme, mitochondrial (CYP11A1),
3 β-hydroxysteroid dehydrogenase/ Δ 5-->4-isomerase type 1 (HSD3B1),
Steroid Delta-isomerase (HSD3B2),
succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial (OXCT1),
mitochondrial D-β-hydroxybutyrate dehydrogenase (BDH1),
cytochrome b5 (CYB5A)

Unification Links: GO:0005739

Relationship Links: Wikipedia:RELATED-TO:Mitochondrion

Report Errors or Provide Feedback
Please cite the following article in publications resulting from the use of MetaCyc: Caspi et al, Nucleic Acids Research 42:D459-D471 2014
Page generated by Pathway Tools version 19.5 (software by SRI International) on Fri Feb 12, 2016, biocyc11.