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ACO2 aconitase 2, mitochondrial [ Homo sapiens (human) ]

Gene ID: 50, updated on 7-Aug-2014
Official Symbol
ACO2provided by HGNC
Official Full Name
aconitase 2, mitochondrialprovided by HGNC
Primary source
HGNC:118
Locus tag
RP3-347H13.8
See related
Ensembl:ENSG00000100412; HPRD:00012; MIM:100850; Vega:OTTHUMG00000030544
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
ICRD; ACONM
Summary
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]
See ACO2 in Epigenomics, MapViewer
Location:
22q13.2
Exon count:
18
Annotation release Status Assembly Chr Location
106 current GRCh38 (GCF_000001405.26) 22 NC_000022.11 (41469125..41528989)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 22 NC_000022.10 (41865099..41924993)

Chromosome 22 - NC_000022.11Genomic Context describing neighboring genes Neighboring gene transducer of ERBB2, 2 Neighboring gene PHD finger protein 5A Neighboring gene polymerase (RNA) III (DNA directed) polypeptide H (22.9kD) Neighboring gene cold shock domain containing C2, RNA binding Neighboring gene phosphomannomutase 1

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

  • 2-Oxocarboxylic acid metabolism, organism-specific biosystem (from KEGG)
    2-Oxocarboxylic acid metabolism, organism-specific biosystem2-Oxocarboxylic acids, also called 2-oxo acids and alpha-keto acids, are the most elementary set of metabolites that includes pyruvate (2-oxopropanoate), 2-oxobutanoate, oxaloacetate (2-oxosuccinate)...
  • 2-Oxocarboxylic acid metabolism, conserved biosystem (from KEGG)
    2-Oxocarboxylic acid metabolism, conserved biosystem2-Oxocarboxylic acids, also called 2-oxo acids and alpha-keto acids, are the most elementary set of metabolites that includes pyruvate (2-oxopropanoate), 2-oxobutanoate, oxaloacetate (2-oxosuccinate)...
  • Biosynthesis of amino acids, organism-specific biosystem (from KEGG)
    Biosynthesis of amino acids, organism-specific biosystemThis map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module fo...
  • Biosynthesis of amino acids, conserved biosystem (from KEGG)
    Biosynthesis of amino acids, conserved biosystemThis map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module fo...
  • Carbon metabolism, organism-specific biosystem (from KEGG)
    Carbon metabolism, organism-specific biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
  • Carbon metabolism, conserved biosystem (from KEGG)
    Carbon metabolism, conserved biosystemCarbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excludi...
  • Citrate cycle (TCA cycle), organism-specific biosystem (from KEGG)
    Citrate cycle (TCA cycle), organism-specific biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
  • Citrate cycle (TCA cycle), conserved biosystem (from KEGG)
    Citrate cycle (TCA cycle), conserved biosystemThe citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form ...
  • Citrate cycle (TCA cycle, Krebs cycle), organism-specific biosystem (from KEGG)
    Citrate cycle (TCA cycle, Krebs cycle), organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Citrate cycle (TCA cycle, Krebs cycle), conserved biosystem (from KEGG)
    Citrate cycle (TCA cycle, Krebs cycle), conserved biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, organism-specific biosystem (from KEGG)
    Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, organism-specific biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, conserved biosystem (from KEGG)
    Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, conserved biosystemPathway module; Carbohydrate and lipid metabolism; Central carbohydrate metabolism
  • Citric acid cycle (TCA cycle), organism-specific biosystem (from REACTOME)
    Citric acid cycle (TCA cycle), organism-specific biosystemIn the citric acid or tricarboxylic acid (TCA) cycle, the acetyl group of acetyl CoA (derived primarily from oxidative decarboxylation of pyruvate, beta-oxidation of long-chain fatty acids, and catab...
  • Glyoxylate and dicarboxylate metabolism, organism-specific biosystem (from KEGG)
    Glyoxylate and dicarboxylate metabolism, organism-specific biosystem
    Glyoxylate and dicarboxylate metabolism
  • Glyoxylate and dicarboxylate metabolism, conserved biosystem (from KEGG)
    Glyoxylate and dicarboxylate metabolism, conserved biosystem
    Glyoxylate and dicarboxylate metabolism
  • Metabolism, organism-specific biosystem (from REACTOME)
    Metabolism, organism-specific biosystemMetabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as th...
  • Metabolism of proteins, organism-specific biosystem (from REACTOME)
    Metabolism of proteins, organism-specific biosystemProtein metabolism comprises the pathways of translation, post-translational modification and protein folding.
  • Mitochondrial protein import, organism-specific biosystem (from REACTOME)
    Mitochondrial protein import, organism-specific biosystemA human mitochondrion contains about 1500 proteins, more than 99% of which are encoded in the nucleus, synthesized in the cytosol and imported into the mitochondrion. Proteins are targeted to four lo...
  • Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystem (from REACTOME)
    Pyruvate metabolism and Citric Acid (TCA) cycle, organism-specific biosystemPyruvate metabolism and the citric acid (TCA) cycle together link the processes of energy metabolism in a human cell with one another and with key biosynthetic reactions. Pyruvate, derived from the r...
  • TCA Cycle, organism-specific biosystem (from WikiPathways)
    TCA Cycle, organism-specific biosystemThe [[wikipedia:citric_acid_cycle|citric acid cycle]], also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, (or rarely, the Szent-Gyorgyi-Krebs cycle) is a series of enzyme-cata...
  • TCA cycle II (eukaryotic), organism-specific biosystem (from BIOCYC)
    TCA cycle II (eukaryotic), organism-specific biosystemGeneral Background The TCA pathway is a catabolic pathway of aerobic respiration. It generates energy and reducing power. It is the first step in generating precursors for biosynthesis. When aceta...
  • TCA cycle VIII (metazoan), conserved biosystem (from BIOCYC)
    TCA cycle VIII (metazoan), conserved biosystemGeneral Background The TCA tricarboxylic acid cycle is a pathway of aerobic respiration that generates both energy and reducing power. It can also generate precursors for various biosynthetic pathwa...
  • The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystem (from REACTOME)
    The citric acid (TCA) cycle and respiratory electron transport, organism-specific biosystemThe metabolism of pyruvate provides one source of acetyl-CoA which enters the citric acid (TCA, tricarboxylic acid) cycle to generate energy and the reducing equivalent NADH. These reducing equivalen...
Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Clone Names

  • MGC20605, MGC33908

Gene Ontology Provided by GOA

Function Evidence Code Pubs
3 iron, 4 sulfur cluster binding IEA
Inferred from Electronic Annotation
more info
 
4 iron, 4 sulfur cluster binding IEA
Inferred from Electronic Annotation
more info
 
aconitate hydratase activity IEA
Inferred from Electronic Annotation
more info
 
iron ion binding IDA
Inferred from Direct Assay
more info
PubMed 
Process Evidence Code Pubs
cell death IEA
Inferred from Electronic Annotation
more info
 
cellular metabolic process TAS
Traceable Author Statement
more info
 
citrate metabolic process IDA
Inferred from Direct Assay
more info
PubMed 
generation of precursor metabolites and energy TAS
Traceable Author Statement
more info
PubMed 
isocitrate metabolic process IEA
Inferred from Electronic Annotation
more info
 
small molecule metabolic process TAS
Traceable Author Statement
more info
 
tricarboxylic acid cycle IDA
Inferred from Direct Assay
more info
PubMed 
tricarboxylic acid cycle IEA
Inferred from Electronic Annotation
more info
 
tricarboxylic acid cycle TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
mitochondrial matrix TAS
Traceable Author Statement
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
 
nucleus IDA
Inferred from Direct Assay
more info
 
Preferred Names
aconitate hydratase, mitochondrial
Names
aconitate hydratase, mitochondrial
citrate hydro-lyase
NP_001089.1

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_032143.1 

    Range
    5001..64865
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001098.2NP_001089.1  aconitate hydratase, mitochondrial precursor

    See proteins identical to NP_001089.1

    Status: REVIEWED

    Source sequence(s)
    AA724140, BC014092, BC026196
    Consensus CDS
    CCDS14017.1
    UniProtKB/Swiss-Prot
    Q99798
    Related
    ENSP00000216254, OTTHUMP00000165920, ENST00000216254, OTTHUMT00000259151
    Conserved Domains (3) summary
    cd01584
    Location:95505
    Blast Score: 2256
    AcnA_Mitochondrial; Aconitase catalyzes the reversible isomerization of citrate and isocitrate as part of the TCA cycle
    TIGR01340
    Location:45775
    Blast Score: 3197
    aconitase_mito; aconitate hydratase, mitochondrial
    cd01578
    Location:587735
    Blast Score: 840
    AcnA_Mitochon_Swivel; Mitochondrial aconitase A swivel domain. Aconitase (also known as aconitate hydratase and citrate hydro-lyase) catalyzes the reversible isomerization of citrate and isocitrate as part of the TCA cycle. This is the aconitase swivel domain, which undergoes ...

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 106

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38 Primary Assembly

Genomic

  1. NC_000022.11 

    Range
    41469125..41528989
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate HuRef

Genomic

  1. AC_000154.1 

    Range
    24830931..24890807
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate CHM1_1.1

Genomic

  1. NC_018933.2 

    Range
    41825012..41884897
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)