ACO1 aconitate hydratase ACO1 [Saccharomyces cerevisiae S288C] - Gene

Summary

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Official Symbol: ACO1
Official Full Name: aconitate hydratase ACO1
Primary source: SGD:S000004295
Locus tag: YLR304C
See related: AllianceGenome:SGD:S000004295; FungiDB:YLR304C; VEuPathDB:YLR304C
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Saccharomyces cerevisiae S288C (strain: S288C)
Lineage: Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Also known as: GLU1
Summary: Enables aconitate hydratase activity and nucleic acid binding activity. Involved in mitochondrial genome maintenance and tricarboxylic acid cycle. Located in cytosol and mitochondrial nucleoid. Used to study central nervous system disease; neurodegenerative disease; optic atrophy 9; and optic nerve disease. 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, Dec 2024]
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Genomic context

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See ACO1 in Genome Data Viewer

Location:: chromosome: XII

Exon count:: 1

Sequence:: Chromosome: XII; NC_001144.5 (735212..737548, complement)

Chromosome XII - NC_001144.5 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_001144.5

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Yeast ACO1 mitochondrial import is modulated by interactions of its C and N terminal domains and SSA1/SSA2.
Title: Yeast aconitase mitochondrial import is modulated by interactions of its C and N terminal domains and Ssa1/2 (Hsp70).
Lack of the ACO1 gene results in increased zinc tolerance.
Title: On the molecular relationships between high-zinc tolerance and aconitase (Aco1) in Saccharomyces cerevisiae.
mitochondria are opportunistic organelles that seize proteins, such as metabolic enzymes, for construction of the nucleoid, an mitochondrial DNA maintenance/segregation apparatus
Title: Yeast aconitase binds and provides metabolically coupled protection to mitochondrial DNA.
essential for mtDNA maintenance independent of its catalytic activity; may integrate metabolic signals and mtDNA maintenance
Title: Aconitase couples metabolic regulation to mitochondrial DNA maintenance.
Yeast aconitase isoenzymes distribute between two distinct subcellular compartments and participate in two separate metabolic pathways: the glyoxylate shunt in the cytosol and the tricarboxylic acid cycle in mitochondria.
Title: Yeast aconitase in two locations and two metabolic pathways: seeing small amounts is believing.
frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation
Title: Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity.

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Pathways from PubChem

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Homology

Orthologs from OrthoDB

Gene Ontology Provided by SGD

Function	Evidence Code	Pubs
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 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	IEA Inferred from Electronic Annotation more info
enables aconitate hydratase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables double-stranded DNA binding	IDA Inferred from Direct Assay more info	PubMed
enables lyase activity	IEA Inferred from Electronic Annotation more info
enables mRNA binding	IDA Inferred from Direct Assay more info	PubMed
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables single-stranded DNA binding	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
involved_in mitochondrial genome maintenance	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in tricarboxylic acid cycle	IBA Inferred from Biological aspect of Ancestor more info
involved_in tricarboxylic acid cycle	IDA Inferred from Direct Assay more info	PubMed
involved_in tricarboxylic acid cycle	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in cytoplasm	IEA Inferred from Electronic Annotation more info
is_active_in cytosol	IBA Inferred from Biological aspect of Ancestor more info
located_in cytosol	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial matrix	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial nucleoid	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	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: aconitate hydratase ACO1

NP_013407.1

EC 4.2.1.3

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

NCBI Reference Sequences (RefSeq)

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Genome Annotation

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

Reference assembly

Genomic

NC_001144.5 Reference assembly

Range

735212..737548 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_001182192.1 → NP_013407.1 TPA: aconitate hydratase ACO1 [Saccharomyces cerevisiae S288C]

See identical proteins and their annotated locations for NP_013407.1

Status: REVIEWED

UniProtKB/Swiss-Prot

D6VYU7, P19414

UniProtKB/TrEMBL

A7A1I8, B3RHI2, C7GUC7, C8ZDR7, G2WJC5, N1P1J9

Conserved Domains (1) summary

TIGR01340
Location:41 → 772

aconitase_mito; aconitate hydratase, mitochondrial