ACO1 / YLR304C Overview

Standard Name
ACO1 1
Systematic Name
Feature Type
ORF , Verified
Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; mutation in human homolog ACO2 is associated with dominant optic nerve atrophy; human homolog ACO2 can complement yeast null mutant 1 2 3 4 5
Name Description
ACOnitase 1
Comparative Info
Sequence Details


The S. cerevisiae Reference Genome sequence is derived from laboratory strain S288C. Download DNA or protein sequence, view genomic context and coordinates. Click "Sequence Details" to view all sequence information for this locus, including that for other strains.

ACO1/YLR304C is located on the right arm of chromosome XII between isoleucine tRNA and STT4 phosphatidylinositol-4-kinase; coding sequence is 2337 nucleotides long with 10 SNPs, one of which causes a Lys/Asn polymorphism at residue 310
Protein Details


Basic sequence-derived (length, molecular weight, isoelectric point) and experimentally-determined (median abundance, median absolute deviation) protein information. Click "Protein Details" for further information about the protein such as half-life, abundance, domains, domains shared with other proteins, protein sequence retrieval for various strains, physico-chemical properties, protein modification sites, and external identifiers for the protein.

Aco1p is 778 amino acids long, moderately abundant, and extremely long-lived; contains 9 aconitase/3-isopropylmalate dehydratase large subunit alpha/beta/alpha domains; methylated on K360, ubiquitinylated on K254, acetylated on 15 lysines, phosphorylated on 19 residues, succinylated on 45 lysines
Length (a.a.)
Mol. Weight (Da)
Isoelectric Point
Median Abundance (molecules/cell)
43808 +/- 22386
Half-life (hr)


Curated mutant alleles for the specified gene, listed alphabetically. Click on the allele name to open the allele page. Click "SGD search" to view all alleles in search results.

View all ACO1 alleles in SGD search

Gene Ontology Details

Gene Ontology

GO Annotations consist of four mandatory components: a gene product, a term from one of the three Gene Ontology (GO) controlled vocabularies (Molecular Function, Biological Process, and Cellular Component), a reference, and an evidence code. SGD has manually curated and high-throughput GO Annotations, both derived from the literature, as well as computational, or predicted, annotations. Click "Gene Ontology Details" to view all GO information and evidence for this locus as well as biological processes it shares with other genes.

Aconitate hydratase with role in tricarboxylic acid (TCA) cycle and mitochondrial genome maintenance; also shows DNA binding activity; localized to mitochondria and cytosol

View computational annotations

Molecular Function

Manually Curated

Biological Process

Manually Curated

Cellular Component

Manually Curated


Phenotype Details


Phenotype annotations for a gene are curated single mutant phenotypes that require an observable (e.g., "cell shape"), a qualifier (e.g., "abnormal"), a mutant type (e.g., null), strain background, and a reference. In addition, annotations are classified as classical genetics or high-throughput (e.g., large scale survey, systematic mutation set). Whenever possible, allele information and additional details are provided. Click "Phenotype Details" to view all phenotype annotations and evidence for this locus as well as phenotypes it shares with other genes.

Non-essential gene; null mutant shows glutamate auxotrophy, sensitivity to hyperosmotic stress, increased accumulation of iron in the mitochondria, loss of mitochondrial genome and deficiency in respiratory growth
Disease Details


Disease Annotations consist of three mandatory components: a gene product, a term from the Disease Ontology (DO) controlled vocabulary and an evidence code. SGD provides manually curated DO Annotations derived from the literature. Click "Disease Details" to view all Disease information and evidence for this locus as well as diseases it shares with other genes.

Yeast ACO1 is homologous to human ACO2, and has been used to study mutations found in patients with neurodegenerative disease, optic nerve disease, and central nervous system disease
Interaction Details


Interaction annotations are curated by BioGRID and include physical or genetic interactions observed between at least two genes. An interaction annotation is composed of the interaction type, name of the interactor, assay type (e.g., Two-Hybrid), annotation type (e.g., manual or high-throughput), and a reference, as well as other experimental details. Click "Interaction Details" to view all interaction annotations and evidence for this locus, including an interaction visualization.

Aco1p interacts physically with proteins involved in mitochondrion organization; ACO1 interacts genetically with genes involved in lipid metabolism and chemical response

320 total interactions for 267 unique genes

Physical Interactions

  • Affinity Capture-MS: 108
  • Affinity Capture-RNA: 12
  • Affinity Capture-Western: 5
  • Co-fractionation: 1
  • Co-localization: 1
  • Cross-Linking-MS (XL-MS): 16
  • PCA: 2
  • Proximity Label-MS: 2
  • Reconstituted Complex: 2

Genetic Interactions

  • Dosage Growth Defect: 2
  • Dosage Lethality: 1
  • Dosage Rescue: 3
  • Negative Genetic: 104
  • Phenotypic Enhancement: 2
  • Phenotypic Suppression: 8
  • Positive Genetic: 39
  • Synthetic Growth Defect: 8
  • Synthetic Lethality: 2
  • Synthetic Rescue: 2
Regulation Details


The number of putative Regulators (genes that regulate it) and Targets (genes it regulates) for the given locus, based on experimental evidence. This evidence includes data generated through high-throughput techniques. Click "Regulation Details" to view all regulation annotations, shared GO enrichment among regulation Targets, and a regulator/target diagram for the locus.

ACO1 encodes mitochondrial aconitase (aconite hydratase), an enzyme of the tricarboxylic acid (TCA) cycle, which is a key part of aerobic respiration, as well as a source of several essential metabolic precursors. In addition to its role in cellular metabolism, Aco1p is also involved in maintenance of mitochondrial DNA (mtDNA). Aco1p localizes to mitochondrial matrix and it is also found in association with protein-DNA complexes called nucleoids that are responsible for packaging of mtDNA. Aco1p binds both double-stranded and single-stranded DNA and protects mtDNA from breaks and recombination, thereby stabilizing the mitochondrial genome. Mutants lacking Aco1p lose mtDNA, whereas constitutively expressed ACO1 rescues the severe mtDNA instability of mutants lacking a key nucleoid component Abf2p. ACO1 is a nuclear gene and its expression is controlled by two regulatory pathways: the glucose-repressed heme-activated HAP system (Hap2p-Hap3p-Hap4p-Hap5p), which controls respiratory activity, and the retrograde (RTG) system (Rtg1p-Rtg3p), which responds to mitochondrial dysfunction. Thus, the dual function of Aco1p appears to provide a direct regulatory link between metabolic signals and mtDNA stability. This signaling mechanism may be preserved in higher eukaryotes, as human homolog ACO2 can complement yeast aco1 null mutants.
Expression Details


Expression data are derived from records contained in the Gene Expression Omnibus (GEO), and are first log2 transformed and normalized. Referenced datasets may contain one or more condition(s), and as a result there may be a greater number of conditions than datasets represented in a single clickable histogram bar. The histogram division at 0.0 separates the down-regulated (green) conditions and datasets from those that are up-regulated (red). Click "Expression Details" to view all expression annotations and details for this locus, including a visualization of genes that share a similar expression pattern.

Summary Paragraph

A summary of the locus, written by SGD Biocurators following a thorough review of the literature. Links to gene names and curated GO terms are included within the Summary Paragraphs.

Last Updated: 2024-07-09

Literature Details


All manually curated literature for the specified gene, organized into topics according to their relevance to the gene (Primary Literature, Additional Literature, or Review). Click "Literature Details" to view all literature information for this locus, including shared literature between genes.