| http://purl.uniprot.org/citations/23475850 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/23475850 | http://www.w3.org/2000/01/rdf-schema#comment | "A decline in α-ketoglutarate dehydrogenase complex (KGDHC) activity has been associated with neurodegeneration. Provision of succinyl-CoA by KGDHC is essential for generation of matrix ATP (or GTP) by substrate-level phosphorylation catalyzed by succinyl-CoA ligase. Here, we demonstrate ATP consumption in respiration-impaired isolated and in situ neuronal somal mitochondria from transgenic mice with a deficiency of either dihydrolipoyl succinyltransferase (DLST) or dihydrolipoyl dehydrogenase (DLD) that exhibit a 20-48% decrease in KGDHC activity. Import of ATP into the mitochondrial matrix of transgenic mice was attributed to a shift in the reversal potential of the adenine nucleotide translocase toward more negative values due to diminished matrix substrate-level phosphorylation, which causes the translocase to reverse prematurely. Immunoreactivity of all three subunits of succinyl-CoA ligase and maximal enzymatic activity were unaffected in transgenic mice as compared to wild-type littermates. Therefore, decreased matrix substrate-level phosphorylation was due to diminished provision of succinyl-CoA. These results were corroborated further by the finding that mitochondria from wild-type mice respiring on substrates supporting substrate-level phosphorylation exhibited ~30% higher ADP-ATP exchange rates compared to those obtained from DLST(+/-) or DLD(+/-) littermates. We propose that KGDHC-associated pathologies are a consequence of the inability of respiration-impaired mitochondria to rely on "in-house" mitochondrial ATP reserves."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.org/dc/terms/identifier | "doi:10.1096/fj.12-220202"xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Beal M.F."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Gibson G.E."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Chinopoulos C."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Doczi J."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Kawamata H."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Manfredi G."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Kiss G."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Zhang S.F."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Konrad C."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Starkov A.A."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/author | "Adam-Vizi V."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/date | "2013"xsd:gYear |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/name | "FASEB J"xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/pages | "2392-2406"xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/title | "The negative impact of alpha-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation."xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://purl.uniprot.org/core/volume | "27"xsd:string |
| http://purl.uniprot.org/citations/23475850 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/23475850 |
| http://purl.uniprot.org/citations/23475850 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/23475850 |
| http://purl.uniprot.org/uniprot/#_A0A140VJQ5-mappedCitation-23475850 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23475850 |
| http://purl.uniprot.org/uniprot/#_A0A024R713-mappedCitation-23475850 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23475850 |
| http://purl.uniprot.org/uniprot/#_E9PCR7-mappedCitation-23475850 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23475850 |
| http://purl.uniprot.org/uniprot/#_E9PEX6-mappedCitation-23475850 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/23475850 |