Hypophosphatasia (HPP) is a rare skeletal disorder caused by loss-of-function mutations in Alkaline Phosphatase, Biomineralization associated (ALPL) gene that encodes tissue-nonspecific alkaline phosphatase. Odontohypophosphatasia (odonto-HPP), a mild form of HPP, is characterized only by oral manifestations including premature exfoliation of deciduous teeth. Enzyme replacement therapy (ERT) is effective in severe HPP cases; however, information about its efficacy for odonto-HPP is limited. A 2-yr-old girl was referred to our hospital for mobility of her deciduous teeth with low serum alkaline phosphatase (ALP) level of 253 U/L (reference range: 410-1,150 U/L) and high urine phosphoethanolamine level of 1,419.9 µmol/g•Cre (7-70 µmol/g•Cre). She had no history of bone fractures; however, several members of her family had low serum ALP levels with a history of pathological fractures. She had a novel heterozygous missense mutation (c.1183A>T, p.Ile395Phe) in ALPL, and therefore, was diagnosed with odonto-HPP. After she was provided ERT to prevent premature exfoliation, no tooth mobility was observed. However, two deciduous teeth exfoliated two months after starting ERT, which was possibly triggered by a bout of common cold. Starting ERT following tooth mobility might be relatively late. Previous studies on experimental mice showed that starting ERT at birth may be effective in preventing premature exfoliation of deciduous teeth.
Tomato [Solanum lycopersicum (formerly Lycopersicon esculentum) L. cv. Momotarou] plants were grown under potted conditions inside the greenhouse of Hiroshima University, Japan. The effects of salinity stress under either ambient or elevated CO 2 atmospheric conditions during the fruit-growth period were compared. Stem was the major sink organ for the carbon assimilates from the source leaves. A simple nondestructive micromorphometric technique was used to measure growth of these organs. The effect of salt stress under ambient air or elevated CO 2 concentration was studied on the apparent photosynthesis (source activity), partitioning 13 C, Na content, and antioxidative enzymes. Compared with the control (without salt), salt-stress treatment severely decreased whole-plant biomass. The treatment also depressed leaf photosynthesis and transport of 13 C assimilates; however, the impact of stress on these activities was alleviated under elevated CO 2 concentration and such alleviation was promoted when sink activity relative to source activity was higher, suggesting that sink activity is involved in alleviation of photosynthesis impaired from salinity. Catalase and ascorbate peroxidase activities increased by salinity stress but they decreased by elevated CO 2 . These results suggested that salinity stress suppresses stem growth in tomato plants; however, the adverse effect is alleviated under elevated CO 2 concentration due not to elevation of source activity in leaves but to activation of sink activity, probably owing to improvement of oxidative stress as well as the water status through stomatal closure at high CO 2 concentration.
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