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Frontiers in Genetics, 6, 157

These observations are all consistent with a defect in mitochondrial iron delivery

Tetrahydrobiopterin (BH 4 ) plays a vital role in this reaction by stabilizing the eNOS dimer and preventing the formation of superoxide, a potentially harmful byproduct, ensuring NO synthesis proceeds efficiently (Xia et al., 1998a

Nuclei (DAPI staining, Abcam PLC, ab104139), and nucleoli (FIB-1::GFP staining) in individual cells were contoured and the corresponding areas were quantified using the ZEISS ZEN Imaging Software