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Targeted Redox Regulation α-Ketoglutarate Dehydrogenase Complex for the Treatment of Human Diseases [PDF]

Cells
α-ketoglutarate dehydrogenase complex (KGDHc) is a crucial enzyme in the tricarboxylic acid (TCA) cycle that intersects monosaccharides, amino acids, and fatty acid catabolism with oxidative phosphorylation (OxPhos). A key feature of KGDHc is its ability
Ryan J. Mailloux
doaj   +4 more sources

The α-ketoglutarate dehydrogenase complex in cancer metabolic plasticity. [PDF]

Cancer Metab, 2017
Deregulated metabolism is a well-established hallmark of cancer. At the hub of various metabolic pathways deeply integrated within mitochondrial functions, the α-ketoglutarate dehydrogenase complex represents a major modulator of electron transport chain activity and tricarboxylic acid cycle (TCA) flux, and is a pivotal enzyme in the metabolic ...
Vatrinet R, Leone G, De Luise M, Girolimetti G, Vidone M, Gasparre G, Porcelli AM.   +6 more
europepmc   +6 more sources

Microgliosis, neuronal death, minor behavioral abnormalities and reduced endurance performance in alpha-ketoglutarate dehydrogenase complex deficient mice [PDF]

Redox Biology
The alpha-ketoglutarate dehydrogenase complex (KGDHc), also known as the 2-oxoglutarate dehydrogenase complex, plays a crucial role in oxidative metabolism.
Márton Kokas, András Budai, Andrea Kádár, Soroosh Mozaffaritabar, Lei Zhou, Tímea Téglás, Rebeka Sára Orova, Dániel Gáspár, Kristóf Németh, Daniel Marton Toth, Nabil V. Sayour, Csenger Kovácsházi, Andrea Xue, Réka Zsuzsanna Szatmári, Beáta Törőcsik, Domokos Máthé, Noémi Kovács, Krisztián Szigeti, Péter Nagy, Ildikó Szatmári, Csaba Fekete, Tamás Arányi, Zoltán V. Varga, Péter Ferdinandy, Zsolt Radák, Andrey V. Kozlov, László Tretter, Tímea Komlódi, Attila Ambrus   +28 more
doaj   +2 more sources

Bioenergetic Signatures of DLD Deficiency: Dissecting PDHc- and α-KGDHc-Linked Defects [PDF]

Antioxidants
Dihydrolipoamide dehydrogenase (DLD) deficiency (MIM #246900) is a rare autosomal recessive mitochondrial disorder caused by pathogenic variants in the DLD gene, which encodes the E3 subunit common to multiple mitochondrial enzyme complexes, including ...
Yarden Haham Zarbib, Shira Huri Ohev-Shalom, Shani Kassia Lyskov, Yuval Mazor, Mika Anekstein-Spigel, Nechama Shalva, Ronen Spiegel, Orna Staretz-Chacham, Joshua Manor, Ann Saada, Rachel Rock, Yair Anikster, Tal Yardeni   +12 more
doaj   +2 more sources

α-Ketoglutarate dehydrogenase complex moonlighting: ROS signalling added to the list: An Editorial highlight for 'Reductions in the mitochondrial enzyme α-ketoglutarate dehydrogenase complex in neurodegenerative disease - beneficial or detrimental?' [PDF]

J Neurochem, 2016
Read the highlighted article ‘Reductions in the mitochondrial enzyme α-ketoglutarate dehydrogenase complex in neurodegenerative disease – beneficial or detrimental?’ on page 823. image
Fernandez E, Bolaños JP.
europepmc   +7 more sources

Inactivation and reactivation of the mitochondrial α-ketoglutarate dehydrogenase complex. [PDF]

J Biol Chem, 2011
Reduced brain metabolism is an invariant feature of Alzheimer Disease (AD) that is highly correlated to the decline in brain functions. Decreased activities of key tricarboxylic acid cycle (TCA) cycle enzymes may underlie this abnormality and are highly correlated to the clinical state of the patient.
Shi Q, Xu H, Yu H, Zhang N, Ye Y, Estevez AG, Deng H, Gibson GE.   +7 more
europepmc   +5 more sources

Parapyruvate Induces Neurodegeneration in C57BL/6JNarl Mice via Inhibition of the α‑Ketoglutarate Dehydrogenase Complex [PDF]

ACS Omega
Inn Lee, Tuzz-Ying Song, Chien-Lin Chen, Jiann-Jou Yang, Nae-Cherng Yang   +4 more
doaj   +2 more sources

Reverse and Forward Electron Flow-Induced H₂O₂ Formation Is Decreased in α-Ketoglutarate Dehydrogenase (α-KGDH) Subunit (E2 or E3) Heterozygote Knock Out Animals

Antioxidants, 2022
α-ketoglutarate dehydrogenase complex (KGDHc), or 2-oxoglutarate dehydrogenase complex (OGDHc) is a rate-limiting enzyme in the tricarboxylic acid cycle, that has been identified in neurodegenerative diseases such as in Alzheimer’s disease.
Gergő Horváth, Gergely Sváb, Tímea Komlódi, Dora Ravasz, Gergely Kacsó, Judit Doczi, Christos Chinopoulos, Attila Ambrus, László Tretter   +8 more
doaj   +1 more source

Overcoming glutamate auxotrophy in Escherichia coli itaconate overproducer by the Weimberg pathway

Metabolic Engineering Communications, 2021
Biosynthesis of itaconic acid occurs through decarboxylation of the TCA cycle intermediate cis-aconitate. Engineering of efficient itaconate producers often requires elimination of the highly active isocitrate dehydrogenase to conserve cis-aconitate ...
Ken W. Lu, Chris T. Wang, Hengray Chang, Ryan S. Wang, Claire R. Shen   +4 more
doaj   +1 more source

Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency

Redox Biology, 2023
The NDUFS4 knockout (KO) mouse phenotype resembles the human Complex I deficiency Leigh Syndrome. The irreversible succination of protein thiols by fumarate is increased in select regions of the NDUFS4 KO brain affected by neurodegeneration.
Gerardo G. Piroli, Allison M. Manuel, Richard S. McCain, Holland H. Smith, Oliver Ozohanics, Sara Mellid, J. Hunter Cox, William E. Cotham, Michael D. Walla, Alberto Cascón, Attila Ambrus, Norma Frizzell   +11 more
doaj   +1 more source