Decreased Nuclear Ascorbate Accumulation Accompanied with Altered Genomic Methylation Pattern in Fibroblasts from Arterial Tortuosity Syndrome Patients

Figure 2

Diminished nuclear accumulation of vitamin C in ATS fibroblasts. Subcellular distribution of ascorbate of human fibroblasts derived from healthy controls or arterial tortuosity syndrome patients were compared by immunoelectron microscopy. After pretreatment of dehydroascorbic acid, cells were fixed and labeled with ascorbate antibody, and analyzed by following immunogold staining. (a) Representative images of the electron microscopy are shown from control (upper panel) and arterial tortuosity syndrome patient (lower panel) samples. Scale bar is 1 μm. N: nucleus, Nu: nucleolus. (b) Nuclear/cytoplasmic ratio of immunogold particles—representing ascorbate—was assessed. A significantly lower level of nucleus/cytoplasm ascorbate ratio was found after dehydroascorbate treatment in fibroblasts derived from arterial tortuosity syndrome patients compared to control fibroblasts (4 in each groups). Data are , .

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