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Studies using skin fibroblasts derived from such Fabry patient biopsies showed a reduced respiratory chain enzyme activity, along with a reduction of energy-rich phosphates AMP, ADP and CP, indicating an impaired mitochondrial energy supply and that this can play a role in the pathogenesis of Fabry disease through a proposed change in the properties of the mitochondria membrane [38], further implicating the mitochondrial dysfunction as part of the symptoms

It is also proposed that aromatic amino acids, such as tryptophan, which are sensitive to oxidation, can be attacked by ROS, such as OH radicals or atomic oxygen (Tolouie et al

qtot 0.266 9 OG311 1 ful O9 9 -0.554 15.9994

To detect lactate concentration secreted by cells, the medium was collected to co-incubate with the working fluid of Lactate Assay Kit-WST (L256, DOJINDO, Japan) for 30 min according to the manufacturers instructions

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