Names & Taxonomy
- Uniprot ID:
- Q99798
- Entry Name:
- ACON_HUMAN
- Status:
- reviewed
- Protein Names:
- Aconitate hydratase, mitochondrial (Aconitase) (EC 4.2.1.3) (Citrate hydro-lyase)
- Gene Names:
- ACO2
- Gene Names Primary:
- ACO2
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 780
- Sequence:
- MAPYSLLVTRLQKALGVRQYHVASVLCQRAKVAMSHFEPNEYIHYDLLEKNINIVRKRLNRPLTLSEKIVYGHLDDPASQEIERGKSYLRLRPDRVAMQDATAQMAMLQFISSGLSKVAVPSTIHCDHLIEAQVGGEKDLRRAKDINQEVYNFLATAGAKYGVGFWKPGSGIIHQIILENYAYPGVLLIGTDSHTPNGGGLGGICIGVGGADAVDVMAGIPWELKCPKVIGVKLTGSLSGWSSPKDVILKVAGILTVKGGTGAIVEYHGPGVDSISCTGMATICNMGAEIGATTSVFPYNHRMKKYLSKTGREDIANLADEFKDHLVPDPGCHYDQLIEINLSELKPHINGPFTPDLAHPVAEVGKVAEKEGWPLDIRVGLIGSCTNSSYEDMGRSAAVAKQALAHGLKCKSQFTITPGSEQIRATIERDGYAQILRDLGGIVLANACGPCIGQWDRKDIKKGEKNTIVTSYNRNFTGRNDANPETHAFVTSPEIVTALAIAGTLKFNPETDYLTGTDGKKFRLEAPDADELPKGEFDPGQDTYQHPPKDSSGQHVDVSPTSQRLQLLEPFDKWDGKDLEDLQILIKVKGKCTTDHISAAGPWLKFRGHLDNISNNLLIGAINIENGKANSVRNAVTQEFGPVPDTARYYKKHGIRWVVIGDENYGEGSSREHAALEPRHLGGRAIITKSFARIHETNLKKQGLLPLTFADPADYNKIHPVDKLTIQGLKDFTPGKPLKCIIKHPNGTQETILLNHTFNETQIEWFRAGSALNRMKELQQ
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.
- Pathway:
- Carbohydrate metabolism; tricarboxylic acid cycle; isocitrate from oxaloacetate: step 2/2.
- Catalytic Activity:
- Citrate = isocitrate.
- Cofactor:
- COFACTOR: Name= cluster; Xref=ChEBI:CHEBI:49883;
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
myelin sheath
nucleus
3 iron, 4 sulfur cluster binding
4 iron, 4 sulfur cluster binding
aconitate hydratase activity
iron ion binding
cellular metabolic process
citrate metabolic process
generation of precursor metabolites and energy
isocitrate metabolic process
small molecule metabolic process
tricarboxylic acid cycle - Gene Ontology Biological Process:
- cellular metabolic process
citrate metabolic process
generation of precursor metabolites and energy
isocitrate metabolic process
small molecule metabolic process
tricarboxylic acid cycle - Gene Ontology Molecular Function:
- 3 iron, 4 sulfur cluster binding
4 iron, 4 sulfur cluster binding
aconitate hydratase activity
iron ion binding - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion
myelin sheath
nucleus - Keywords:
- 4Fe-4S
Acetylation
Complete proteome
Direct protein sequencing
Disease mutation
Iron
Iron-sulfur
Isopeptide bond
Lyase
Metal-binding
Mitochondrion
Neurodegeneration
Phosphoprotein
Polymorphism
Pyrrolidone carboxylic acid
Reference proteome
Transit peptide
Tricarboxylic acid cycle
Ubl conjugation