| Id |
Subject |
Object |
Predicate |
Lexical cue |
| T1 |
0-101 |
Sentence |
denotes |
Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption. |
| T2 |
102-255 |
Sentence |
denotes |
Mitochondrial encephalomyopathy and lactic acidosis with strokelike episodes (MELAS) is a severe young onset stroke disorder without effective treatment. |
| T3 |
256-375 |
Sentence |
denotes |
We have identified a MELAS patient harboring a 13528A-->G mitochondrial DNA (mtDNA) mutation in the Complex I ND5 gene. |
| T4 |
376-475 |
Sentence |
denotes |
This mutation was homoplasmic in mtDNA from patient muscle and nearly homoplasmic (99.9%) in blood. |
| T5 |
476-652 |
Sentence |
denotes |
Fibroblasts from the patient exhibited decreased mitochondrial membrane potential (Deltapsim) and increased lactate production, consistent with impaired mitochondrial function. |
| T6 |
653-926 |
Sentence |
denotes |
Transfer of patient mtDNA to a new nuclear background using transmitochondrial cybrid fusions confirmed the pathogenicity of the 13528A-->G mutation; Complex I-linked respiration and Deltapsim were both significantly reduced in patient mtDNA cybrids compared with controls. |
| T7 |
927-1175 |
Sentence |
denotes |
Inhibition of the adenine nucleotide translocase or the F1F0-ATPase with bongkrekic acid or oligomycin caused a loss of potential in patient mtDNA cybrid mitochondria, indicating a requirement for glycolytically generated ATP to maintain Deltapsim. |
| T8 |
1176-1335 |
Sentence |
denotes |
This was confirmed by inhibition of glycolysis with 2-deoxy-D-glucose, which caused depletion of ATP and mitochondrial depolarization in patient mtDNA cybrids. |
| T9 |
1336-1556 |
Sentence |
denotes |
These data suggest that in response to impaired respiration due to the mtDNA mutation, mitochondria consume ATP to maintain Deltapsim, representing a potential pathophysiological mechanism in human mitochondrial disease. |
