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Handbook of Vitamin C Research

Handbook of Vitamin C Research

Handbook of Vitamin C Research

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380Akihito IshigamiFigure 3. <strong>Vitamin</strong> C biosynthesis pathway. The pathway from D-glucose to L-gulonic acid is sharedwith that <strong>of</strong> early steps in the uronic acid cycle. X is a conjugating molecule for glucuronidation.SMP30 is a gluconolactonase (GNL), which catalyzes from L-gulonic acid to L-gulono-γ-lactone. Inhumans, L-gulonolactone oxidase (GLO) is absent because <strong>of</strong> mutation.Figure 4. Osteogenic disorder <strong>of</strong> SMP30/GNL knockout mice. X-ray images show the skeletalstructures <strong>of</strong> SMP30/GNL knockout and wild type mice. Insets: enlargements <strong>of</strong> the femoral region inwhich an arrow points to the distal femur fracture <strong>of</strong> an SMP30/GNL knockout mouse. Arrowheadsindicate a rachitic rosary at the junction <strong>of</strong> costae and costal cartilage.physiological functions <strong>of</strong> the body as time passes. Yet our SMP30/GNL knockout mice diedearly (as measured by survival times), as previously stated, about four times faster than their

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