Aco2 aconitase 2, mitochondrial [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Aco2provided by MGI
Official Full Name: aconitase 2, mitochondrialprovided by MGI
Primary source: MGI:MGI:87880
See related: Ensembl:ENSMUSG00000022477 AllianceGenome:MGI:87880
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: Aco3; Irp1; Aco-2; D10Wsu183e
Summary: Enables aconitate hydratase activity. Involved in tricarboxylic acid cycle. Located in mitochondrion. Is active in mitochondrial matrix. Is expressed in several structures, including alimentary system; central nervous system; genitourinary system; integumental system; and sensory organ. Human ortholog(s) of this gene implicated in infantile cerebellar-retinal degeneration and optic atrophy 9. Orthologous to human ACO2 (aconitase 2). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in heart adult (RPKM 787.3), kidney adult (RPKM 344.6) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 15 E1; 15 38.26 cM

Exon count:: 18

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	15	NC_000081.7 (81756664..81799338)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	15	NC_000081.6 (81872463..81915137)

Chromosome 15 - NC_000081.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

ACO2 deficiency increases vulnerability to Parkinson's disease via dysregulating mitochondrial function and histone acetylation-mediated transcription of autophagy genes.
Title: ACO2 deficiency increases vulnerability to Parkinson's disease via dysregulating mitochondrial function and histone acetylation-mediated transcription of autophagy genes.
Mitochondrial Aconitase ACO2 Links Iron Homeostasis with Tumorigenicity in Non-Small Cell Lung Cancer.
Title: Mitochondrial Aconitase ACO2 Links Iron Homeostasis with Tumorigenicity in Non-Small Cell Lung Cancer.
Sialylation of Asparagine 612 Inhibits Aconitase Activity during Mouse Sperm Capacitation; a Possible Mechanism for the Switch from Oxidative Phosphorylation to Glycolysis.
Title: Sialylation of Asparagine 612 Inhibits Aconitase Activity during Mouse Sperm Capacitation; a Possible Mechanism for the Switch from Oxidative Phosphorylation to Glycolysis.
NO-mediated suppression of metabolism to mitochondrial ACO2.
Title: Nitric oxide orchestrates metabolic rewiring in M1 macrophages by targeting aconitase 2 and pyruvate dehydrogenase.
Activation of mitochondrial aconitase is acetylation-dependent.
Title: Lysine Acetylation Activates Mitochondrial Aconitase in the Heart.
Ogg1 chaperoning of Aco-2 in preventing oxidant-mediated mtDNA damage and apoptosis may afford an innovative target for the molecular events underlying oxidant-induced toxicity.
Title: Mitochondria-targeted Ogg1 and aconitase-2 prevent oxidant-induced mitochondrial DNA damage in alveolar epithelial cells.
aconitase activity can be regulated by at least two mechanisms: oxidation/reduction and phosphorylation/dephosphorylation
Title: Activation of aconitase in mouse fast-twitch skeletal muscle during contraction-mediated oxidative stress.
IRP-1,-2 are indispensable for regulation of mammalian iron homeostasis at the post-implantation stage of murine embryonic development
Title: Complete loss of iron regulatory proteins 1 and 2 prevents viability of murine zygotes beyond the blastocyst stage of embryonic development.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (2)
Gene trapped (2)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

Aco2 (e-PCR), detects polymorphism
- UniSTS:506703

Aco2 (e-PCR), detects polymorphism
- UniSTS:141004

D10Wsu183e (e-PCR), detects polymorphism
- UniSTS:141527

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 3 iron, 4 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables 3 iron, 4 sulfur cluster binding	ISO Inferred from Sequence Orthology more info
enables 4 iron, 4 sulfur cluster binding	IBA Inferred from Biological aspect of Ancestor more info
enables 4 iron, 4 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables 4 iron, 4 sulfur cluster binding	ISO Inferred from Sequence Orthology more info
enables aconitate hydratase activity	IBA Inferred from Biological aspect of Ancestor more info
enables aconitate hydratase activity	IDA Inferred from Direct Assay more info	PubMed
enables aconitate hydratase activity	ISO Inferred from Sequence Orthology more info
enables iron ion binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within citrate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in citrate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in isocitrate metabolic process	IEA Inferred from Electronic Annotation more info
involved_in isocitrate metabolic process	ISO Inferred from Sequence Orthology more info
involved_in liver development	IEA Inferred from Electronic Annotation more info
involved_in response to isolation stress	IEA Inferred from Electronic Annotation more info
involved_in tricarboxylic acid cycle	IBA Inferred from Biological aspect of Ancestor more info
involved_in tricarboxylic acid cycle	IEA Inferred from Electronic Annotation more info
involved_in tricarboxylic acid cycle	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within tricarboxylic acid cycle	ISO Inferred from Sequence Orthology more info
involved_in tricarboxylic acid cycle	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
is_active_in cytosol	IBA Inferred from Biological aspect of Ancestor more info
is_active_in mitochondrial matrix	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in myelin sheath	HDA more info	PubMed

General protein information

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Preferred Names: aconitate hydratase, mitochondrial

Names: citrate hydro-lyase

NP_542364.1

EC 4.2.1.3

NCBI Reference Sequences (RefSeq)

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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.

mRNA and Protein(s)

NM_080633.2 → NP_542364.1 aconitate hydratase, mitochondrial precursor

See identical proteins and their annotated locations for NP_542364.1

Status: VALIDATED

Source sequence(s)

BI987806, BQ031215, BY732916, CA750686, CO426953, CO429053

Consensus CDS

CCDS27675.1

UniProtKB/Swiss-Prot

Q3UDK9, Q3ULG9, Q3UNH7, Q505P4, Q99KI0

Related

ENSMUSP00000023116.7, ENSMUST00000023116.7

Conserved Domains (3) summary

cd01584
Location:95 → 505

AcnA_Mitochondrial; Aconitase catalyzes the reversible isomerization of citrate and isocitrate as part of the TCA cycle

cd01578
Location:587 → 735

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 ...

TIGR01340
Location:45 → 775

aconitase_mito; aconitate hydratase, mitochondrial