ACOT9 Gene

HGNC Family	Acyl-CoA thioesterases (ACOT)
Name	acyl-CoA thioesterase 9
Description	The protein encoded by this gene is a mitochondrial acyl-CoA thioesterase of unknown function. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\n ACOT9 is a mitochondrial acyl‐CoA thioesterase that plays a central role in regulating the balance between acyl‐CoA, free fatty acids, and CoA within the mitochondria. Detailed kinetic studies have shown that ACOT9 exhibits unusual substrate specificity, displaying high maximal activity with short‐chain substrates (such as propionyl‐CoA and (iso)‐butyryl‐CoA) and a high catalytic efficiency toward saturated long‐chain acyl‐CoAs. This dual substrate recognition enables ACOT9 to simultaneously hydrolyze both short‐ and long‐chain acyl‐CoAs, thereby providing a novel regulatory link between fatty acid and amino acid metabolism in mitochondria."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n Comparative analyses of mitochondrial acyl‐CoA thioesterases revealed that ACOT9 is highly expressed in tissues such as brown adipose tissue and kidney, where it constitutes a major enzyme activity responsible for hydrolyzing saturated C2–C20‐CoA species."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}]}, {"type": "t", "text": " In addition, genomic studies have identified rare variants in ACOT9 that associate with a reduced risk for hyperlipidaemia, underscoring its emerging role in systemic lipid regulation and energy metabolism."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": "\n "}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n Further extending its functional impact, loss‐of‐function studies in mice indicate that hepatic ACOT9 expression directs metabolic fluxes by preferentially deactivating short‐chain fatty acyl‐CoAs at the inner mitochondrial membrane. This activity channels acetyl‐CoA into the tricarboxylic acid cycle, thereby exacerbating de novo lipogenesis and hepatic glucose production under conditions of excessive nutrition, which contributes to the development of nonalcoholic fatty liver disease and steatohepatitis."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": " Moreover, ACOT9 has been implicated in the regulation of energy metabolism and intramuscular fat deposition during muscle development in chickens, highlighting its broader role in metabolic homeostasis."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "5"}]}, {"type": "t", "text": "\n "}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n Collectively, these findings establish ACOT9 as a key mitochondrial enzyme that fine‐tunes metabolic processes by modulating acyl‐CoA levels, thereby influencing fatty acid oxidation, amino acid metabolism, and overall energy balance.\n "}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Veronika Tillander, Elisabet Arvidsson Nordström, Jenny Reilly, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Acyl-CoA thioesterase 9 (ACOT9) in mouse may provide a novel link between fatty acid and amino acid metabolism in mitochondria."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Mol Life Sci (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s00018-013-1422-1"}], "href": "https://doi.org/10.1007/s00018-013-1422-1"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23864032"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23864032"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Carmen Bekeova, Lauren Anderson-Pullinger, Kevin Boye, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Multiple mitochondrial thioesterases have distinct tissue and substrate specificity and CoA regulation, suggesting unique functional roles."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.RA119.010901"}], "href": "https://doi.org/10.1074/jbc.RA119.010901"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31676684"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31676684"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "David Curtis "}, {"type": "b", "children": [{"type": "t", "text": "Analysis of exome-sequenced UK Biobank subjects implicates genes affecting risk of hyperlipidaemia."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Genet Metab (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ymgme.2020.07.009"}], "href": "https://doi.org/10.1016/j.ymgme.2020.07.009"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32747172"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32747172"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Sandra Steensels, Jixuan Qiao, Yanzhen Zhang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Acyl-Coenzyme A Thioesterase 9 Traffics Mitochondrial Short-Chain Fatty Acids Toward De Novo Lipogenesis and Glucose Production in the Liver."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hepatology (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/hep.31409"}], "href": "https://doi.org/10.1002/hep.31409"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32498134"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32498134"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Zhuang Liu, Ning Yang, Yiyuan Yan, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Genome-wide association analysis of egg production performance in chickens across the whole laying period."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "BMC Genet (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/s12863-019-0771-7"}], "href": "https://doi.org/10.1186/s12863-019-0771-7"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31412760"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31412760"}]}]}]}
Synonyms	MT-ACT48, MTACT48, ACATE2, CGI-16
Proteins	ACOT9_HUMAN
NCBI Gene ID	23597
API
Download Associations
Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

ACOT9 has 6,393 functional associations with biological entities spanning 9 categories (molecular profile, organism, chemical, functional term, phrase or reference, disease, phenotype or trait, structural feature, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 108 datasets.

Click the + buttons to view associations for ACOT9 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

ACOT9 Gene

Functional Associations

Please acknowledge the Harmonizome in your publications by citing the following reference(s):

	Dataset	Summary
	Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of ACOT9 gene relative to other tissues from the Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles	tissues with high or low expression of ACOT9 gene relative to other tissues from the Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray	tissue samples with high or low expression of ACOT9 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq	tissue samples with high or low expression of ACOT9 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq dataset.
	Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of ACOT9 gene relative to other tissues from the Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles dataset.
	BioGPS Cell Line Gene Expression Profiles	cell lines with high or low expression of ACOT9 gene relative to other cell lines from the BioGPS Cell Line Gene Expression Profiles dataset.
	BioGPS Human Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of ACOT9 gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	BioGPS Mouse Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of ACOT9 gene relative to other cell types and tissues from the BioGPS Mouse Cell Type and Tissue Gene Expression Profiles dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of ACOT9 gene relative to other cell lines from the CCLE Cell Line Gene CNV Profiles dataset.
	CCLE Cell Line Gene Expression Profiles	cell lines with high or low expression of ACOT9 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with ACOT9 protein from the CCLE Cell Line Proteomics dataset.
	CellMarker Gene-Cell Type Associations	cell types associated with ACOT9 gene from the CellMarker Gene-Cell Type Associations dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of ACOT9 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of ACOT9 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets dataset.
	ChEA Transcription Factor Targets 2022	transcription factors binding the promoter of ACOT9 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	CMAP Signatures of Differentially Expressed Genes for Small Molecules	small molecule perturbations changing expression of ACOT9 gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing ACOT9 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing ACOT9 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores	cellular components containing ACOT9 protein in low- or high-throughput protein localization assays from the COMPARTMENTS Experimental Protein Localization Evidence Scores dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores 2025	cellular components containing ACOT9 protein in low- or high-throughput protein localization assays from the COMPARTMENTS Experimental Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025	cellular components co-occuring with ACOT9 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of ACOT9 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with ACOT9 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with ACOT9 gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with ACOT9 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DepMap CRISPR Gene Dependency	cell lines with fitness changed by ACOT9 gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores 2025	diseases co-occuring with ACOT9 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
	DisGeNET Gene-Disease Associations	diseases associated with ACOT9 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at ACOT9 gene from the ENCODE Histone Modification Site Profiles dataset.
	ENCODE Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of ACOT9 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of ACOT9 gene in ChIP-seq datasets from the ENCODE Transcription Factor Targets dataset.
	ESCAPE Omics Signatures of Genes and Proteins for Stem Cells	PubMedIDs of publications reporting gene signatures containing ACOT9 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of ACOT9 gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneSigDB Published Gene Signatures	PubMedIDs of publications reporting gene signatures containing ACOT9 from the GeneSigDB Published Gene Signatures dataset.
	GEO Signatures of Differentially Expressed Genes for Diseases	disease perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Diseases dataset.
	GEO Signatures of Differentially Expressed Genes for Gene Perturbations	gene perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Gene Perturbations dataset.
	GEO Signatures of Differentially Expressed Genes for Kinase Perturbations	kinase perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Kinase Perturbations dataset.
	GEO Signatures of Differentially Expressed Genes for Small Molecules	small molecule perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Small Molecules dataset.
	GEO Signatures of Differentially Expressed Genes for Transcription Factor Perturbations	transcription factor perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Transcription Factor Perturbations dataset.
	GEO Signatures of Differentially Expressed Genes for Viral Infections	virus perturbations changing expression of ACOT9 gene from the GEO Signatures of Differentially Expressed Genes for Viral Infections dataset.