Successful Management of a Young Athlete with Type 2 Long QT Syndrome by Genotype-specific Risk Stratification and Bridging Therapy with a Wearable Cardioverter Defibrillator.

We herein report a 14-year-old boy with repetitive nocturnal syncope related to medication-refractory long QT syndrome (LQTS). Although the use of an implantable cardioverter-defibrillator (ICD) was inevitable to prevent sudden cardiac death, he refused immediate implantation in order to play in a baseball competition six weeks away. Given his genetic diagnosis of type 2 LQTS, which is associated with cardiac events unrelated to exercise, we prescribed a wearable cardioverter defibrillator (WCD) to be donned at night, without limiting his exercise participation. An ICD was implanted after the competition. We successfully performed the preplanned treatment while maximizing the patient's quality-of-life with a WCD and genotype-specific risk stratification.

Steady sucrose degradation is a prerequisite for tolerance to root hypoxia.

We investigated the role of glycolysis and sucrolysis in the difference in tolerance to root hypoxia between two Myrtaceae tree species, Melaleuca cajuputi (which shows superior tolerance to root hypoxia) and Eucalyptus camaldulensis (which does not). Analysis of the adenylate energy charge (AEC) in roots subjected to a 4-day hypoxic treatment (HT) in hydroponic culture revealed that the interspecies difference in tolerance corresponds to the ability to maintain energy status under root hypoxia: AEC was reduced by HT in E. camaldulensis, but not in M. cajuputi. The energy status in HT roots of E. camaldulensis was restored by feeding of glucose (Glc) but not sucrose (Suc). These data provide evidence that low substrate availability for glycolysis resulting from an impairment of sucrolysis suppresses ATP production under hypoxic conditions in this species. Measurements of the rates of O2 consumption and CO2 production in roots indicated that E. camaldulensis, but not M. cajuputi, failed to activate fermentation in HT roots. These results cannot be attributed to enzymatic dysfunction, because no inhibition of main glycolytic and fermentative enzymes was observed in both species, and Glc feeding had a beneficial effect on AEC of HT roots of E. camaldulensis. The impairment of sucrolysis was demonstrated by inhibited soluble acid invertase activity in HT roots of E. camaldulensis. In contrast, there was no inhibition in all sucrolytic enzymes tested in HT roots of M. cajuputi, suggesting that steady Suc degradation is essential for maintaining high energy status under root hypoxia. We conclude that root sucrolysis is one of the essential factors that determines the extent of tolerance to root hypoxia.

Identification of a hydrolyzable tannin, oenothein B, as an aluminum-detoxifying ligand in a highly aluminum-resistant tree, Eucalyptus camaldulensis.

Eucalyptus camaldulensis is a tree species in the Myrtaceae that exhibits extremely high resistance to aluminum (Al). To explore a novel mechanism of Al resistance in plants, we examined the Al-binding ligands in roots and their role in Al resistance of E. camaldulensis. We identified a novel type of Al-binding ligand, oenothein B, which is a dimeric hydrolyzable tannin with many adjacent phenolic hydroxyl groups. Oenothein B was isolated from root extracts of E. camaldulensis by reverse-phase high-performance liquid chromatography and identified by nuclear magnetic resonance and mass spectrometry analyses. Oenothein B formed water-soluble or -insoluble complexes with Al depending on the ratio of oenothein B to Al and could bind at least four Al ions per molecule. In a bioassay using Arabidopsis (Arabidopsis thaliana), Al-induced inhibition of root elongation was completely alleviated by treatment with exogenous oenothein B, which indicated the capability of oenothein B to detoxify Al. In roots of E. camaldulensis, Al exposure enhanced the accumulation of oenothein B, especially in EDTA-extractable forms, which likely formed complexes with Al. Oenothein B was localized mostly in the root symplast, in which a considerable amount of Al accumulated. In contrast, oenothein B was not detected in three Al-sensitive species, comprising the Myrtaceae tree Melaleuca bracteata, Populus nigra, and Arabidopsis. Oenothein B content in roots of five tree species was correlated with their Al resistance. Taken together, these results suggest that internal detoxification of Al by the formation of complexes with oenothein B in roots likely contributes to the high Al resistance of E. camaldulensis.

Photosynthesis and photoassimilate transport during root hypoxia in Melaleuca cajuputi, a flood-tolerant species, and in Eucalyptus camaldulensis, a moderately flood-tolerant species.

We compared the photosynthetic and photoassimilate transport responses of Melaleuca cajuputi Powell seedlings to root hypoxia with those of Eucalyptus camaldulensis Dehnh. Control and hypoxia treated roots were maintained in a nutrient solution through which air or nitrogen was bubbled. Under root hypoxic conditions, seedlings of M. cajuputi, a flood-tolerant species, maintained height growth, whereas seedlings of E. camaldulensis, a moderately flood-tolerant species, showed markedly decreased height growth compared with control seedlings. Root hypoxia caused decreases in whole-plant biomass, photosynthetic rate and stomatal conductance in E. camaldulensis, but not in M. cajuputi. Photoassimilate transport to roots decreased significantly in E. camaldulensis seedlings 4 days after treatment and starch accumulated in mature leaves. Photoassimilate supply to hypoxic roots of E. camaldulensis seedlings was, thus, limited by reduced photoassimilate transport rather than by reduced photosynthesis. In contrast, M. cajuputi seedlings showed sustained photoassimilate transport to hypoxic roots and persistent photosynthesis, which together provided a substantial photoassimilate supply to the roots. Sucrose accumulated in hypoxic E. camaldulensis roots, but not in hypoxic M. cajuputi roots. A stable, low sucrose concentration in hypoxic roots would let M. cajuputi seedlings prolong photoassimilate transport to the roots. Photoassimilate partitioning among the water-soluble carbohydrates, starch and structural carbohydrates within the roots was unaffected by root hypoxia in E. camaldulensis, but in M. cajuputi, partitioning was shifted somewhat from structural carbohydrates to water-soluble carbohydrates. This suggests that M. cajuputi seedlings are able to increase photoassimilate utilization in metabolism and sustain energy production under root hypoxic conditions.

Citrate-release-mediated aluminum resistance is coupled to the inducible expression of mitochondrial citrate synthase gene in Paraserianthes falcataria.

Aluminum (Al) resistance in some leguminous plants is achieved by enhanced citrate release from roots. Enhancement requires several hours for complete activation and is postulated to involve Al-responsive genes or components. We examined the mechanism of Al-induced citrate release by studying the relationship between citrate release and expression of the mitochondrial citrate synthase (mCS) gene in three leguminous trees. Root elongation in Leucaena leucocephala (Lam.) de Wit was arrested within 24 h by 30 microM Al, whereas root elongation in Paraserianthes falcataria (L.) Neilson and Acacia mangium Willd. was inhibited < 50% by a 48-h treatment with 100 microM Al in calcium chloride solution. Roots of P. falcataria and A. mangium maintained enhanced release and accumulation of citrate for at least 28 days in response to Al treatment. Aluminum increased the accumulation of mCS transcripts in P. falcataria roots, but not in L. leucocephala roots, and thus up-regulation decreased following removal of Al. Lanthanum did not alter the expression level of mCS. Aluminum increased mCS activity concomitantly with enhanced mCS gene expression in P. falcataria, whereas it did not affect mCS activity in L. leucocephala. Aluminum content in root apices of P. falcataria was increased by cycloheximide, supporting the idea that de novo synthesis of proteins is a prerequisite for Al resistance. Our findings suggest that Al-inducible expression of mCS coupled with enhanced citrate release mediates Al resistance in P. falcataria.

Elevated atmospheric CO2 concentration alters the effect of phosphate supply on growth of Japanese red pine (Pinus densiflora) seedlings.

We demonstrated that the inorganic phosphate (P(i)) requirement for growth of Japanese red pine (Pinus densiflora Sieb. & Zucc.) seedlings is increased by elevated CO(2) concentration ([CO(2)]) and that responses of the ectomycorrhizal fungus Pisolithus tinctorius (Pers.) Coker & Couch to P(i) supply are also altered. To investigate the growth response of non-mycorrhizal seedlings to P(i) supply in elevated [CO(2)], non-mycorrhizal seedlings were grown for 73 days in ambient or elevated [CO(2)] (350 or 700 micromol mol(-1)) with nutrient solutions containing one of seven phosphate concentrations (0, 0.02, 0.04, 0.06, 0.08, 0.10 and 0.20 mM). In ambient [CO(2)], the growth response to P(i) was saturated at about 0.1 mM P(i), whereas in elevated [CO(2)], the growth response to P(i) supply did not saturate, even at the highest P(i) supply (0.2 mM), indicating that the P(i) requirement is higher in elevated [CO(2)] than in ambient [CO(2)]. The increased requirement was due mainly to an altered shoot growth response to P(i) supply. The enhanced P(i) requirement in elevated [CO(2)] was not associated with a change in photosynthetic response to P(i) or a change in leaf phosphorus (P) status. We investigated the effect of P(i) supply (0.04, 0.08 and 0.20 mM) on the ectomycorrhizal fungus P. tinctorius in mycorrhizal seedlings grown in ambient or elevated [CO(2)]. Root ergosterol concentration (an indicator of fungal biomass) decreased with increasing P(i) supply in ambient [CO(2)], but the decrease was far less in elevated [CO(2)]. In ambient [CO(2)] the ratio of extramatrical mycelium to root biomass decreased with increasing P(i) supply but did not change in elevated [CO(2)]. We conclude that, because elevated [CO(2)] increased the P(i) requirement for shoot growth, the significance of the ectomycorrhizal association was also increased in elevated [CO(2)].