Aco2 Gene Summary [Mouse]
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, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Aco2 |
| Official Name | aconitase 2, mitochondrial [Source:MGI Symbol;Acc:MGI:87880] |
| Ensembl ID | ENSMUSG00000022477 |
| Bio databases IDs | |
| Aliases | aconitase 2, mitochondrial |
| Synonyms | Aco3, ACON, aconitase 2, Aconitase 2, mitochondrial, Aconitate Hydratase, Mitochondrial, ACONM, D10Wsu183e, HEL-S-284, ICRD, Irp1, LOC105369160, Mitochondrial Aconitase, OCA8, OPA9 |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Aco2 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- aconitate hydratase, putative, Aquifex type
- Aconitase_swivel
- Aconitase C-terminal domain
- binding protein
- homoaconitate hydratase family protein
- Aconitase family (aconitate hydratase)
- iron ion binding
- enzyme
- aconitase catalytic domain
- protein binding
- aconitate hydratase
- aconitate hydratase, mitochondrial
Pathways
Biological processes and signaling networks where the Aco2 gene in mouse plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- schizophrenia
- Alzheimer disease
- infantile cerebellar-retinal degeneration
- mitochondrial disorder
- respiratory disorder
- neoplasia
- optic atrophy type 9
- allergic pulmonary eosinophilia
- autosomal recessive optic atrophy
- hereditary disorder
regulates
- adenosine triphosphate
- TP53
- lipid
- mitochondrial DNA
- citric acid
- oleic acid
- palmitic acid
- isocitric acid
role in cell
- apoptosis
- growth
- biogenesis
- damage in
- biogenesis in
- lipogenesis in
- generation in
- adipogenesis in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- myelin enriched fraction
- cellular membrane
- Mitochondria
- synaptic membrane
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Aco2 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- generation of precursor metabolites and energy
- liver development
- citrate metabolic process
- response to isolation stress
- isocitrate metabolic process
- tricarboxylic acid cycle
Cellular Component
Where in the cell the gene product is active
- mitochondrial matrix
- mitochondrion
- cytosol
Molecular Function
What the gene product does at the molecular level
- iron ion binding
- 3 iron, 4 sulfur cluster binding
- 4 iron, 4 sulfur cluster binding
- aconitate hydratase activity