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    OGDH oxoglutarate dehydrogenase [ Homo sapiens (human) ]

    Gene ID: 4967, updated on 4-Jan-2025

    GeneRIFs: Gene References Into Functions

    GeneRIFPubMed TitleDate
    OGDH and Bcl-xL loss causes synthetic lethality in glioblastoma.

    OGDH and Bcl-xL loss causes synthetic lethality in glioblastoma.
    Nguyen TT, Torrini C, Shang E, Shu C, Mun JY, Gao Q, Humala N, Akman HO, Zhang G, Westhoff MA, Karpel-Massler G, Bruce JN, Canoll P, Siegelin MD., Free PMC Article

    05/7/2024
    Biallelic variants in OGDH encoding oxoglutarate dehydrogenase lead to a neurodevelopmental disorder characterized by global developmental delay, movement disorder, and metabolic abnormalities.

    Biallelic variants in OGDH encoding oxoglutarate dehydrogenase lead to a neurodevelopmental disorder characterized by global developmental delay, movement disorder, and metabolic abnormalities.
    Whittle EF, Chilian M, Karimiani EG, Progri H, Buhas D, Kose M, Ganetzky RD, Toosi MB, Torbati PN, Badv RS, Shelihan I, Yang H, Elloumi HZ, Lee S, Jamshidi Y, Pittman AM, Houlden H, Ignatius E, Rahman S, Maroofian R, Yoon WH, Carroll CJ., Free PMC Article

    02/28/2023
    A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease.

    A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease.
    Yap ZY, Strucinska K, Matsuzaki S, Lee S, Si Y, Humphries K, Tarnopolsky MA, Yoon WH., Free PMC Article

    12/25/2021
    DHTKD1 and OGDH display substrate overlap in cultured cells and form a hybrid 2-oxo acid dehydrogenase complex in vivo.

    DHTKD1 and OGDH display substrate overlap in cultured cells and form a hybrid 2-oxo acid dehydrogenase complex in vivo.
    Leandro J, Dodatko T, Aten J, Nemeria NS, Zhang X, Jordan F, Hendrickson RC, Sanchez R, Yu C, DeVita RJ, Houten SM., Free PMC Article

    08/7/2021
    Synthetic analogues of 2-oxo acids discriminate metabolic contribution of the 2-oxoglutarate and 2-oxoadipate dehydrogenases in mammalian cells and tissues.

    Synthetic analogues of 2-oxo acids discriminate metabolic contribution of the 2-oxoglutarate and 2-oxoadipate dehydrogenases in mammalian cells and tissues.
    Artiukhov AV, Grabarska A, Gumbarewicz E, Aleshin VA, Kähne T, Obata T, Kazantsev AV, Lukashev NV, Stepulak A, Fernie AR, Bunik VI., Free PMC Article

    11/21/2020
    there is the direct interaction between SIRT5 and 2-oxoglutarate dehydrogenase (OGDH), and desuccinylation of OGDH by SIRT5 inhibits the activity of OGDH complex.

    OGDH mediates the inhibition of SIRT5 on cell proliferation and migration of gastric cancer.
    Lu X, Yang P, Zhao X, Jiang M, Hu S, Ouyang Y, Zeng L, Wu J.

    06/13/2020
    The data indicate that amino acid/nucleotide metabolism-related genes OGDH, PPAT and PCCA acquire somatic mutations in microsatellite instability-high gastric cancers and colorectal cancers and that mutational intratumoral heterogeneity may occur in at least some of these tumors.

    Frameshift mutations of OGDH, PPAT and PCCA genes in gastric and colorectal cancers.
    Jo YS, Oh HR, Kim MS, Yoo NJ, Lee SH.

    02/2/2019
    PIK3CA mutant cancer cells require PIK3CA but also require the expression of the TCA cycle enzyme 2-oxoglutarate dehydrogenase (OGDH).

    PIK3CA mutant tumors depend on oxoglutarate dehydrogenase.
    Ilic N, Birsoy K, Aguirre AJ, Kory N, Pacold ME, Singh S, Moody SE, DeAngelo JD, Spardy NA, Freinkman E, Weir BA, Tsherniak A, Cowley GS, Root DE, Asara JM, Vazquez F, Widlund HR, Sabatini DM, Hahn WC., Free PMC Article

    04/28/2018
    oxoglutarate dehydrogenase (OGDH) and lipoic acid synthase (LIAS), which when mutated stabilize HIF1alpha in a non-hydroxylated form.

    Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1α Stability in Aerobic Conditions.
    Burr SP, Costa AS, Grice GL, Timms RT, Lobb IT, Freisinger P, Dodd RB, Dougan G, Lehner PJ, Frezza C, Nathan JA., Free PMC Article

    07/1/2017
    Tissue-specific expression of OGDH splice variants may thus provide a mechanism that tunes the control of the enzyme to the specialized metabolic and signalling needs of individual cell types

    Calcium-insensitive splice variants of mammalian E1 subunit of 2-oxoglutarate dehydrogenase complex with tissue-specific patterns of expression.
    Denton RM, Pullen TJ, Armstrong CT, Heesom KJ, Rutter GA., Free PMC Article

    06/10/2017
    ATP consumption is demonstrated in respiration-impaired isolated mice and in situ neuronal mitochondria from transgenic mice with dihydrolipoyl succinyltransferase deficiency.

    The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation.
    Kiss G, Konrad C, Doczi J, Starkov AA, Kawamata H, Manfredi G, Zhang SF, Gibson GE, Beal MF, Adam-Vizi V, Chinopoulos C., Free PMC Article

    08/3/2013
    Acute inhibition of alpha-ketoglutarate dehydrogenase produces effects on calcium opposite to those in Alzheimer's disease (AD), while the chronic or long-term inhibition of alpha-KGDHC mimicked the AD-related changes in calcium.

    Deficits in the mitochondrial enzyme α-ketoglutarate dehydrogenase lead to Alzheimer's disease-like calcium dysregulation.
    Gibson GE, Chen HL, Xu H, Qiu L, Xu Z, Denton TT, Shi Q., Free PMC Article

    01/26/2013
    Differences in oxoglutarate dehydrogenase activity suggest that there is no excess mitochondrial capacity during maximal exercise with a small muscle mass.

    Exercise training induces similar elevations in the activity of oxoglutarate dehydrogenase and peak oxygen uptake in the human quadriceps muscle.
    Blomstrand E, Krustrup P, Søndergaard H, Rådegran G, Calbet JA, Saltin B.

    12/3/2011
    Observational study of gene-disease association. (HuGE Navigator)

    Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
    Hendrickson SL, Lautenberger JA, Chinn LW, Malasky M, Sezgin E, Kingsley LA, Goedert JJ, Kirk GD, Gomperts ED, Buchbinder SP, Troyer JL, O'Brien SJ., Free PMC Article

    12/5/2010
    Association with autosomal recessive DOOR syndrome not found.

    Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome.
    van Bever Y, Balemans W, Duval EL, Jespers A, Eyskens F, van Hul W, Courtens W.

    01/21/2010
    2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability is regulated by the RING finger ubiquitin ligase Siah

    Regulation of 2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability by the RING finger ubiquitin ligase Siah.
    Habelhah H, Laine A, Erdjument-Bromage H, Tempst P, Gershwin ME, Bowtell DD, Ronai Z.

    01/21/2010
    Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity.

    Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity.
    Shi Q, Chen HL, Xu H, Gibson GE.

    01/21/2010
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