OGDHL Gene

Name	oxoglutarate dehydrogenase-like
Description	The protein encoded by this gene is similar to oxoglutarate dehydrogenase (OGDH) of the OGDH complex, which degrades glucose and glutamate. This gene encodes several isoforms, including some that appear to localize to mitochondria. The encoded protein down-regulates the AKT signaling cascade and can suppress the growth of cervical cancer cells. [provided by RefSeq, Dec 2016]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nOxoglutarate dehydrogenase‐like (OGDHL) is a rate‐limiting component of the α‐ketoglutarate dehydrogenase complex, with a critical role in the tricarboxylic acid cycle and mitochondrial metabolism. Multiple studies in cancer contexts show that OGDHL is frequently downregulated—often by promoter hypermethylation or loss‐of-function mutations—which disrupts normal mitochondrial function and metabolic homeostasis. Reduced OGDHL activity can result in diminished reactive oxygen species (ROS) production that otherwise would help trigger apoptosis via caspase‐3 activation and downregulate survival pathways such as AKT signaling. This loss of function is associated with increased cell proliferation, invasion, and metastasis in diverse tumors including cervical, colorectal, pancreatic, liver, and breast cancers; in hepatocellular carcinoma, for example, decreased OGDHL levels promote the accumulation of L‑2‑hydroxyglutarate, stabilize HIF‑1α, and drive epithelial–mesenchymal transition."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "6"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its tumor‐suppressive roles, bi-allelic pathogenic variants in OGDHL have been linked to a spectrum of neurological and neurodevelopmental disorders. Functional models using CRISPR-Cas9–mediated knockouts in Drosophila and studies in human neuronal cells have demonstrated that loss of OGDHL perturbs the Krebs cycle and impairs mitochondrial respiration. These metabolic deficiencies are associated with clinical features such as epilepsy, hearing loss, visual impairment, gait ataxia, and structural brain anomalies including microcephaly and hypoplasia of the corpus callosum, underscoring the enzyme’s indispensable role in neurodevelopment."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Tanusree Sen, Nilkantha Sen, Maartje G Noordhuis, et al. "}, {"type": "b", "children": [{"type": "t", "text": "OGDHL is a modifier of AKT-dependent signaling and NF-κB function."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0048770"}], "href": "https://doi.org/10.1371/journal.pone.0048770"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23152800"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23152800"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Mohammad Khalaj-Kondori, Mina Hosseinnejad, Asghar Hosseinzadeh, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Aberrant hypermethylation of OGDHL gene promoter in sporadic colorectal cancer."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Curr Probl Cancer (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.currproblcancer.2019.03.001"}], "href": "https://doi.org/10.1016/j.currproblcancer.2019.03.001"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30904169"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30904169"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Yao Liu, Fanzheng Meng, Jiabei Wang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "A Novel Oxoglutarate Dehydrogenase-Like Mediated miR-214/TWIST1 Negative Feedback Loop Inhibits Pancreatic Cancer Growth and Metastasis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Clin Cancer Res (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1158/1078-0432.CCR-18-4113"}], "href": "https://doi.org/10.1158/1078-0432.CCR-18-4113"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31175094"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31175094"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Yan Jiao, Yanqing Li, Zhuo Fu, et al. "}, {"type": "b", "children": [{"type": "a", "children": [{"type": "t", "text": "i"}], "href": "i"}, {"type": "t", "text": "OGDHL"}, {"type": "a", "children": [{"type": "t", "text": "/i"}], "href": "/i"}, {"type": "t", "text": " Expression as a Prognostic Biomarker for Liver Cancer Patients."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Dis Markers (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1155/2019/9037131"}], "href": "https://doi.org/10.1155/2019/9037131"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31781311"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31781311"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Xingyi Guo, Jirong Long, Zhishan Chen, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Discovery of rare coding variants in OGDHL and BRCA2 in relation to breast cancer risk in Chinese women."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Int J Cancer (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/ijc.32825"}], "href": "https://doi.org/10.1002/ijc.32825"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "31837001"}], "href": "https://pubmed.ncbi.nlm.nih.gov/31837001"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Weiqi Dai, Yueyue Li, Weijie Sun, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Silencing of OGDHL promotes liver cancer metastasis by enhancing hypoxia inducible factor 1 α protein stability."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cancer Sci (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1111/cas.15540"}], "href": "https://doi.org/10.1111/cas.15540"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "36000493"}], "href": "https://pubmed.ncbi.nlm.nih.gov/36000493"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Zheng Yie Yap, Stephanie Efthymiou, Simone Seiffert, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Bi-allelic variants in OGDHL cause a neurodevelopmental spectrum disease featuring epilepsy, hearing loss, visual impairment, and ataxia."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Hum Genet (2021)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ajhg.2021.11.003"}], "href": "https://doi.org/10.1016/j.ajhg.2021.11.003"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "34800363"}], "href": "https://pubmed.ncbi.nlm.nih.gov/34800363"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Sheng-Jia Lin, Barbara Vona, Tracy Lau, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Evaluating the association of biallelic OGDHL variants with significant phenotypic heterogeneity."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Genome Med (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/s13073-023-01258-4"}], "href": "https://doi.org/10.1186/s13073-023-01258-4"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38031187"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38031187"}]}]}]}
Proteins	OGDHL_HUMAN
NCBI Gene ID	55753
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

OGDHL has 5,517 functional associations with biological entities spanning 8 categories (molecular profile, organism, disease, phenotype or trait, functional term, phrase or reference, chemical, structural feature, cell line, cell type or tissue, gene, protein or microRNA) extracted from 106 datasets.

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

If available, associations are ranked by standardized value

Harmonizome 3.0

OGDHL 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 OGDHL 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 OGDHL gene relative to other tissues from the Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles	tissue samples with high or low expression of OGDHL gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray	tissue samples with high or low expression of OGDHL 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 OGDHL 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 OGDHL 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 OGDHL 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 OGDHL gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of OGDHL 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 OGDHL gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with OGDHL protein from the CCLE Cell Line Proteomics dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of OGDHL gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of OGDHL 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 OGDHL gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	ClinVar Gene-Phenotype Associations 2025	phenotypes associated with OGDHL gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing OGDHL protein from integrated AP-MS and IF data from the CM4AI U2OS Cell Map Protein Localization Assemblies dataset.
	CMAP Signatures of Differentially Expressed Genes for Small Molecules	small molecule perturbations changing expression of OGDHL gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing OGDHL protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores 2025	cellular components containing OGDHL protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
	COMPARTMENTS Experimental Protein Localization Evidence Scores 2025	cellular components containing OGDHL 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 OGDHL 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 OGDHL gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with OGDHL gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Chemical Interactions	chemicals interacting with OGDHL gene/protein from the curated CTD Gene-Chemical Interactions dataset.
	CTD Gene-Disease Associations	diseases associated with OGDHL gene/protein from the curated CTD Gene-Disease Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of OGDHL protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DepMap CRISPR Gene Dependency	cell lines with fitness changed by OGDHL gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with OGDHL gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with OGDHL gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores 2025	diseases co-occuring with OGDHL 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 OGDHL gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with OGDHL gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at OGDHL 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 OGDHL gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of OGDHL 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 OGDHL from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GAD Gene-Disease Associations	diseases associated with OGDHL gene in GWAS and other genetic association datasets from the GAD Gene-Disease Associations dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of OGDHL gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneRIF Biological Term Annotations	biological terms co-occuring with OGDHL gene in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.
	GeneSigDB Published Gene Signatures	PubMedIDs of publications reporting gene signatures containing OGDHL from the GeneSigDB Published Gene Signatures dataset.