Gene Details

ID's — ACO2 ; Q99798
Alias SwissProt Name Old Accessions Ensembl Gene Description
ACON_HUMAN O75809,Q5JZ41,Q6FHX0,Q8TAQ6 ENSG00000100412 Aconitate hydratase, mitochondrial

Features
Chromosome location Molecular wt. (Da) Protein length
22q13.2 85425 780

Properties
Subcellular
Localization		 Enzyme ID Domain Function Disease
Mitochondrion 4.2.1.3 --- Catalyzes the isomerization of citrate to isocitrate via cis-aconitate. {ECO:0000250|UniProtKB:P16276}. Optic atrophy 9 (OPA9) [MIM:616289]: A condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts. {ECO:0000269|PubMed:25351951}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Classification of DNLCs into distinct cell subtypes (i.e. neuronal or glial)
  Neurons Oligodendrocyte
progenitor cells		 Neuron
progenitor cells		 Microglial cells
Transcript
expression count		 490 80 98 152
Average UMI count
(unique molecular identifier)		 1.47 1.55 1.01 0.94
Cell subtype Oligodendrocyte precursor cells FDR(q value) 9.44341E-13

Mitochondrial localization
Evidence
MitoCarta v2.0|GO|GeneCards: 5|PMID: 23371551

Complexes

Complex details — ACO2 ; Q99798
Complex Id
 Number of subunits Complex members View Complex
CID002 31 PARK7, HINT2, CLIC4, CLIC1, NDUFAB1, DDAH1, PGLS, DDAH2, SOD1, ADA, ENO1, GSTP1, UCHL1, GCSH, MPST, PRDX6, C21orf33, CMPK1, NOS2, PGM1, ARCN1, IDH2, HINT1, TPI1, LACTB2, ACO2, HDHD3, C1orf198, PDP1, GRHPR, NENF  

Interactions

PPI — ACO2 ; Q99798 interacts with
Protein1
 Protein2
 Gene1
 Gene2
 Is Mt
Protein1		 Is Mt
Protein2		 Celltype
Protein1		 Celltype
Protein2		 Σ LLS
DN Score		 Σ LLS
ECSC Score		 Fold Change DF
Interaction		 p-Value DF Interactions
 # Sources of supporting evidence Description1
 Description2
Q99798 Q9NX46 ACO2 ADPRHL2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- --- Aconitate hydratase, mitochondrial Poly(ADP-ribose) glycohydrolase ARH3
Q99798 Q9Y696 ACO2 CLIC4 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Aconitate hydratase, mitochondrial Chloride intracellular channel protein 4
Q99798 Q9HC38 ACO2 GLOD4 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Aconitate hydratase, mitochondrial Glyoxalase domain-containing protein 4
Q99798 Q9UBQ7 ACO2 GRHPR Y Y Oligodendrocyte precursor cells Neurons --- --- --- --- Y Aconitate hydratase, mitochondrial Glyoxylate reductase/hydroxypyruvate reductase
Q99798 Q9BSH5 ACO2 HDHD3 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Aconitate hydratase, mitochondrial Haloacid dehalogenase-like hydrolase domain-containing protein 3
Q99798 Q9BX68 ACO2 HINT2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Aconitate hydratase, mitochondrial Histidine triad nucleotide-binding protein 2, mitochondrial
Q99798 Q9UMX5 ACO2 NENF Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- --- Aconitate hydratase, mitochondrial Neudesin
Q99798 Q9NQR4 ACO2 NIT2 Y Y Oligodendrocyte precursor cells Neurons --- 1.58 --- --- Y Aconitate hydratase, mitochondrial Omega-amidase NIT2
Q99798 Q9P0J1 ACO2 PDP1 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- 1.48 4.343e+0 Y Aconitate hydratase, mitochondrial [Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial
Q99798 Q9Y2S7 ACO2 POLDIP2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Aconitate hydratase, mitochondrial Polymerase delta-interacting protein 2
P54819 Q99798 AK2 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- 1.77 --- --- Y Adenylate kinase 2, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03168};Adenylate kinase 2, mitochondrial, N-terminally processed {ECO:0000255|HAMAP-Rule:MF_03168} Aconitate hydratase, mitochondrial
P30038 Q99798 ALDH4A1 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells 1.46 --- 1.45 2.568e+0 Y Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial Aconitate hydratase, mitochondrial
P48444 Q99798 ARCN1 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- --- Coatomer subunit delta Aconitate hydratase, mitochondrial
P30042 Q99798 C21orf33 ACO2 Y Y --- Oligodendrocyte precursor cells --- 1.31 --- --- Y ES1 protein homolog, mitochondrial Aconitate hydratase, mitochondrial
P62158 Q99798 CALM ACO2 Y Y --- Oligodendrocyte precursor cells --- --- 1.2 2.530e+0 --- Calmodulin Aconitate hydratase, mitochondrial
O00299 Q99798 CLIC1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- 1.78 5.019e+0 Y Chloride intracellular channel protein 1 Aconitate hydratase, mitochondrial
P30085 Q99798 CMPK1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- --- UMP-CMP kinase {ECO:0000255|HAMAP-Rule:MF_03172} Aconitate hydratase, mitochondrial
Q96BR5 Q99798 COA7 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Cytochrome c oxidase assembly factor 7 Aconitate hydratase, mitochondrial
P06733 Q99798 ENO1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- Y Alpha-enolase Aconitate hydratase, mitochondrial
O95571 Q99798 ETHE1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- 1.16 -2.022e+0 Y Persulfide dioxygenase ETHE1, mitochondrial Aconitate hydratase, mitochondrial
P10109 Q99798 FDX1 ACO2 Y Y --- Oligodendrocyte precursor cells --- --- 1.71 2.637e+0 Y Adrenodoxin, mitochondrial Aconitate hydratase, mitochondrial
Q6P4F2 Q99798 FDX2 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- 1.69 2.160e+0 Y Ferredoxin-2, mitochondrial {ECO:0000303|PubMed:20547883} Aconitate hydratase, mitochondrial
P23434 Q99798 GCSH ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- 1.94 4.022e+0 Y Glycine cleavage system H protein, mitochondrial {ECO:0000303|PubMed:1671321} Aconitate hydratase, mitochondrial
Q86SX6 Q99798 GLRX5 ACO2 Y Y --- Oligodendrocyte precursor cells --- --- --- --- Y Glutaredoxin-related protein 5, mitochondrial Aconitate hydratase, mitochondrial
P06744 Q99798 GPI ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Glucose-6-phosphate isomerase Aconitate hydratase, mitochondrial
P78417 Q99798 GSTO1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- Y Glutathione S-transferase omega-1 Aconitate hydratase, mitochondrial
P09211 Q99798 GSTP1 ACO2 Y Y --- Oligodendrocyte precursor cells --- --- --- --- Y Glutathione S-transferase P Aconitate hydratase, mitochondrial
P25325 Q99798 MPST ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y 3-mercaptopyruvate sulfurtransferase Aconitate hydratase, mitochondrial
O14561 Q99798 NDUFAB1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- Y Acyl carrier protein, mitochondrial Aconitate hydratase, mitochondrial
Q99497 Q99798 PARK7 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- Y Protein/nucleic acid deglycase DJ-1 {ECO:0000305|PubMed:25416785, ECO:0000305|PubMed:28596309} Aconitate hydratase, mitochondrial
O95336 Q99798 PGLS ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- --- 6-phosphogluconolactonase Aconitate hydratase, mitochondrial
P36871 Q99798 PGM1 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Phosphoglucomutase-1 Aconitate hydratase, mitochondrial
P30041 Q99798 PRDX6 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- 1.87 3.561e+0 Y Peroxiredoxin-6 Aconitate hydratase, mitochondrial
P22307 Q99798 SCP2 ACO2 Y Y Neurons Oligodendrocyte precursor cells 1.72 --- 1.6 2.131e+0 Y Non-specific lipid-transfer protein Aconitate hydratase, mitochondrial
P00441 Q99798 SOD1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- Y Superoxide dismutase [Cu-Zn] Aconitate hydratase, mitochondrial
Q5T1C6 Q99798 THEM4 ACO2 Y Y Oligodendrocyte precursor cells Oligodendrocyte precursor cells --- --- --- --- Y Acyl-coenzyme A thioesterase THEM4 Aconitate hydratase, mitochondrial
P60174 Q99798 TPI1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- 1.8 2.196e+0 Y Triosephosphate isomerase Aconitate hydratase, mitochondrial
O43396 Q99798 TXNL1 ACO2 Y Y --- Oligodendrocyte precursor cells --- --- --- --- --- Thioredoxin-like protein 1 Aconitate hydratase, mitochondrial
P09936 Q99798 UCHL1 ACO2 Y Y Neurons Oligodendrocyte precursor cells --- --- --- --- --- Ubiquitin carboxyl-terminal hydrolase isozyme L1 Aconitate hydratase, mitochondrial