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- W2394534095 abstract "Hereditary spastic paraplegia (HSP) is a group of familial neurodegenerative disorders characterized by progressive lower limb spasticity and weakness due to degeneration of corticospinal axons. These disorders are classified both genetically, according to the mode of inheritance, and clinically, as pure and complicated forms. Recently the discovery that paraplegin, the defective protein in autosomal recessive HSP-SPG7, localizes in mitochondria allowed to foster the hypothesis that some mitochondrial dysfunctions can play a pathogenic role in HSP. The aim of our study was to indirectly evaluate oxidative metabolism in contracting mu scle, by assessing the anaerobic lactate threshold in 7 patients (5 M and 2 F, mean age 48.0±13.9 yrs) affected by HSP, both autosomal dominant or sporadic, during an incremental bicycle exercise. Analysis of venous lactate curve showed that lactate levels were significantly higher than in controls (peak normalised lactate: 378.8 vs. 271%, p<0.01). Furthermore, an early threshold of lactate was detected only in HSP patients. Even if other factors such as chronic spasticity or muscle deconditioning have to be taken into account in the interpretation of our data, these results suggest possible occurrence of mitochondrial involvement in skeletal muscle of HSP patients." @default.
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- W2394534095 date "2000-01-01" @default.
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- W2394534095 title "Altered lactate kinetics from exercising muscle in Hereditary Spastic Paraplegia." @default.
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