TY - GEN AU - Piroli, Gerardo G AU - Manuel, Allison M AU - McCain, Richard S AU - Smith, Holland H AU - Ozohanics, Oliver AU - Mellid, Sara AU - Cox, J Hunter AU - Cotham, William E AU - Walla, Michael D AU - Cascon Soriano, Alberto AU - Ambrus, Attila AU - Frizzell, Norma PY - 2023 UR - https://hdl.handle.net/20.500.12105/26050 AB - 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. We report... LA - eng PB - Elsevier KW - Alpha-ketoglutarate dehydrogenase KW - Complex I KW - Fumarate KW - Leigh syndrome KW - Protein succination KW - Substrate level phosphorylation TI - Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency. TY - research article ER -