ArticleNeurobiology of disease2009
Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins.
Article in Neurobiology of disease, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 28 citations in OpenAlex.
- DLST-driven metabolic alterations link skin to blood glucose: A translational study from mouse to human.Molecular metabolism · 2026Article
- Protein lipoylation in cancer: metabolic reprogramming and therapeutic potential.Cell death discovery · 2025Review
- Mitochondrial Alpha-Keto Acid Dehydrogenase Complexes: Recent Developments on Structure and Function in Health and Disease.Sub-cellular biochemistry · 2024Review
- Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency.Redox biology · 2023Article
- Mitochondrial heterogeneity in diseases.Signal transduction and targeted therapy · 2023Review
- The α-Ketoglutarate Dehydrogenase Complex as a Hub of Plasticity in Neurodegeneration and Regeneration.International journal of molecular sciences · 2022Review
- Reverse and Forward Electron Flow-Induced HAntioxidants (Basel, Switzerland) · 2022Article
- Myocardial disturbances of intermediary metabolism in Barth syndrome.Frontiers in cardiovascular medicine · 2022Review
- Inhibition of 2-Oxoglutarate Dehydrogenase as a Chemical Model of Acute Hypobaric Hypoxia.Frontiers in medicine · 2021Article
- Review
- Recurrent Germline DLST Mutations in Individuals with Multiple Pheochromocytomas and Paragangliomas.American journal of human genetics · 2019Article
- α-ketoglutarate dehydrogenase inhibition counteracts breast cancer-associated lung metastasis.Cell death & disease · 2018Article
- Reductions in the mitochondrial enzyme α-ketoglutarate dehydrogenase complex in neurodegenerative disease - beneficial or detrimental?Journal of neurochemistry · 2016Article
- Mild mitochondrial metabolic deficits by α-ketoglutarate dehydrogenase inhibition cause prominent changes in intracellular autophagic signaling: Potential role in the pathobiology of Alzheimer's disease.Neurochemistry international · 2016Article
- Novel Metabolic Abnormalities in the Tricarboxylic Acid Cycle in Peripheral Cells From Huntington's Disease Patients.PloS one · 2016Article
- The RNA-binding protein HuR is essential for the B cell antibody response.Nature immunology · 2015Article
- Investigation on the Origin of Sperm DNA Fragmentation: Role of Apoptosis, Immaturity and Oxidative Stress.Molecular medicine (Cambridge, Mass.) · 2015Article
- Xanthoceraside ameliorates mitochondrial dysfunction contributing to the improvement of learning and memory impairment in mice with intracerebroventricular injection of aβ1-42.Evidence-based complementary and alternative medicine : eCAM · 2014Article
- An update on the role of mitochondrial α-ketoglutarate dehydrogenase in oxidative stress.Molecular and cellular neurosciences · 2013Review
- The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2013Article
Corrections and comments
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Authors and funding
12 authors at 2 institutions in 1 country.
Funding
Abstract
The activity of a key mitochondrial tricarboxylic acid cycle enzyme, alpha-ketoglutarate dehydrogenase complex (KGDHC), declines in many neurodegenerative diseases. KGDHC consists of three subunits. The dihydrolipoyl succinyl transferase (DLST) component is unique to KGDHC. DLST(+/-) mice showed reduced mRNA and protein levels and decreased brain mitochondrial KGDHC activity. Neurotoxic effects of mitochondrial toxins were exacerbated in DLST(+/-) mice. MPTP produced a significantly greater reduction of striatal dopamine and tyrosine hydroxylase-positive neurons in the substantia nigra pars compacta of DLST(+/-) mice. DLST deficiency enhanced the severity of lipid peroxidation in the substantia nigra after MPTP treatment. Striatal lesions induced by either malonate or 3-nitropropionic acid (3-NP) were significantly larger in DLST(+/-) mice than in wildtype controls. DLST deficiency enhanced the 3-NP inhibition of mitochondria enzymes, and 3-NP induced protein and DNA oxidations. These observations support the hypothesis that reductions in KGDHC may impair the adaptability of the brain and contribute to the pathogenesis of neurodegenerative diseases.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.