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

Gene ID: 50, updated on 7-Dec-2014
Official Symbol
ACO2provided by HGNC
Official Full Name
aconitase 2, mitochondrialprovided by HGNC
Primary source
HGNC: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?

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
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
    AcnA_Mitochondrial; Aconitase catalyzes the reversible isomerization of citrate and isocitrate as part of the TCA cycle
    TIGR01340
    Location:45775
    aconitase_mito; aconitate hydratase, mitochondrial
    cd01578
    Location:587735
    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 CHM1_1.1

Genomic

  1. NC_018933.2 

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

Alternate HuRef

Genomic

  1. AC_000154.1 

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