NP_001089.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
aconitate hydratase, mitochondrial
NCBI Official Synonym Full Names
aconitase 2
NCBI Official Synonym Symbols
ICRD; OCA8; OPA9; ACONM; HEL-S-284 [Similar Products]
NCBI Protein Information
aconitate hydratase, mitochondrial
UniProt Protein Name
Aconitate hydratase, mitochondrial
UniProt Synonym Protein Names
Citrate hydro-lyase
UniProt Synonym Gene Names
NCBI Summary for ACO2
The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq, Jul 2008]
UniProt Comments for ACO2
ACO2: Catalyzes the isomerization of citrate to isocitrate via cis-aconitate. Monomer. Belongs to the aconitase/IPM isomerase family.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glyoxylate and dicarboxylate; EC 4.2.1.3; Lyase; Mitochondrial
Chromosomal Location of Human Ortholog: 22q13.2
Cellular Component: mitochondrial matrix; mitochondrion; myelin sheath
Molecular Function: 3 iron, 4 sulfur cluster binding; 4 iron, 4 sulfur cluster binding; aconitate hydratase activity; iron ion binding
Biological Process: citrate metabolic process; generation of precursor metabolites and energy; isocitrate metabolic process; liver development; tricarboxylic acid cycle
Disease: Infantile Cerebellar-retinal Degeneration; Optic Atrophy 8
Research Articles on ACO2
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Pathways associated with anti-ACO2 antibody
Diseases associated with anti-ACO2 antibody
Organs/Tissues associated with anti-ACO2 antibody
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