-The radial growth of mature cork oaks (Quercus suber L.) was studied during a 9-year production cycle using monthly dendrometer measurements and cork ring widths. The mean tree radial increase was 5.1 mm yr -1 and the cork increment accounting for 3.8 mm yr -1 . The radial growth curves for cork, wood and stem were very similar, showing a decreasing trend along the production cycle. Cork increment was always the largest fraction of tree growth (on average 74%) and showed less inter-annual variations as compared to wood. Tree radial growth presented a clear within-the-year seasonal pattern, extending from March to October, with a maximum in JuneJuly. The overall pattern of monthly growth distribution was similar among the years of the production cycle, but some inter-annual variations occurred with 1-2 month shifts or monthly growth rate decreases, related to climatic factors. Early spring growth was enhanced by winter rain, autumn growth by high summer rain and June increments by high temperatures during this month. cork oak / diameter growth / cork growth / climatic fluctuations / dendroclimatology Résumé -Influence du climat sur la variabilité saisonnière de la croissance radiale du chêne-liège au cours d'un cycle de production de liège. La croissance radiale de chênes-liège (Quercus suber L.) adultes en pleine production a été suivie pendant un cycle de production de 9 ans par des mesures mensuelles de dendromètres et par la mesure des cernes du liège. L'accroissement annuel des arbres a été de 5,1 mm an -1 , dont 3,8 mm an -1 de liège. Les courbes de croissance du liège, du bois et de la tige ont montré une même tendance à la diminution de la croissance au cours du cycle. Le liège, qui correspond à la fraction majeure de l'accroissement de l'arbre (en moyenne 74 %), a montré une variation interannuelle de croissance moindre que le bois. La croissance soumise à une variation saisonnière marquée, s'étend de mars à octobre, avec un maximum en juin-juillet. La distribution de la croissance a été similaire toutes les années, parfois avec des déphasages de 1 à 2 mois ou avec une diminution de l'accroissement résultant de variations interannuelles de précipita-tion ou de température. La croissance au début du printemps a été favorisée par les pluies d'hiver, la croissance en automne par les pluies d'été et les accroissements en juin par une température moyenne plus élevée pendant ce mois.chêne-liège / croissance radiale / croissance du liège / influences climatiques / dendroclimatologie Ann. For. Sci. 59 (2002) [429][430][431][432][433][434][435][436][437] 429

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The diameter growth of mature cork oaks under cork production in southwestern Portugal was studied during a 9-year cork-production cycle in relation to fluctuations in precipitation and temperature, and to drought indices combining the two variables. A dendroclimatological approach was applied to the series of 8 complete years contained in the cork-production cycle and growth effects were removed by standardization and calculation of annual growth indices. The most important factor positively influencing diameter growth was cumulative precipitation in the growing season (January-June) and in the previous autumn and winter (October/November of the previous year-June). The monthly temperature is a less influential factor, negatively correlated with diameter growth in high summer drought (August) and positively correlated at the beginning of physiological activity (March).

Background The pyruvate dehydrogenase complex (PDC) catalyzes the irreversible decarboxylation of pyruvate into acetyl-CoA. PDC deficiency can be caused by alterations in any of the genes encoding its several subunits. The resulting phenotype, though very heterogeneous, mainly affects the central nervous system. The aim of this study is to describe and discuss the clinical, biochemical and genotypic information from thirteen PDC deficient patients, thus seeking to establish possible genotype–phenotype correlations. Results The mutational spectrum showed that seven patients carry mutations in the PDHA1 gene encoding the E1α subunit, five patients carry mutations in the PDHX gene encoding the E3 binding protein, and the remaining patient carries mutations in the DLD gene encoding the E3 subunit. These data corroborate earlier reports describing PDHA1 mutations as the predominant cause of PDC deficiency but also reveal a notable prevalence of PDHX mutations among Portuguese patients, most of them carrying what seems to be a private mutation (p.R284X). The biochemical analyses revealed high lactate and pyruvate plasma levels whereas the lactate/pyruvate ratio was below 16; enzymatic activities, when compared to control values, indicated to be independent from the genotype and ranged from 8.5% to 30%, the latter being considered a cut-off value for primary PDC deficiency. Concerning the clinical features, all patients displayed psychomotor retardation/developmental delay, the severity of which seems to correlate with the type and localization of the mutation carried by the patient. The therapeutic options essentially include the administration of a ketogenic diet and supplementation with thiamine, although arginine aspartate intake revealed to be beneficial in some patients. Moreover, in silico analysis of the missense mutations present in this PDC deficient population allowed to envisage the molecular mechanism underlying these pathogenic variants. Conclusion The identification of the disease-causing mutations, together with the functional and structural characterization of the mutant protein variants, allow to obtain an insight on the severity of the clinical phenotype and the selection of the most appropriate therapy.

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