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Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri-du-chat syndrome. The sample consisted of 10 children and adolescents with Cri-du-chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile - Revised. An observational protocol was used to record adaptive and maladaptive behaviours. The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule-following. These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri-du-chat syndrome. [ABSTRACT FROM AUTHOR]

Cereport™ (RMP-7) is a selective bradykinin B2 receptor agonist which increases the permeability of the 'blood-brain tumour barrier' (BBTB) to increase delivery of chemotherapeutic agents to brain tumours. A series of experiments was performed in an RG2 rodent model of glioma to evaluate and refine intravenous (i.v.) parameters to optimize Cereport's clinical utility. The first experiment demonstrated that while carboplatin levels were increased by twofold when given as a bolus during the Cereport infusion, no increase in carboplatin levels were seen when Cereport and carboplatin were simultaneously co-infused for 15 min. A subsequent experiment established that a major factor responsible for the lack of an effect with the co-infusion paradigm was tachyphylaxis to Cereport during the 15 min infusion, for a progressively diminished response to Cereport occurred over that time frame, as plasma levels of carboplatin were rising. A final experiment adjusted the timing of the Cereport and carboplatin infusions so that higher plasma carboplatin levels were achieved prior to initiating the Cereport infusion. Significant uptake effects were achieved when the carboplatin infusion preceded the Cereport infusion by 10 min (i.e. 5 min overlap in the delivery of the two agents). Collectively, these data provide the first systematic evaluation of dosing parameters involving receptor-mediated changes in BBTB permeability and provide new information regarding the pharmacodynamics and potential clinical use of Cereport. [ABSTRACT FROM AUTHOR]

Exchange rates of CO2 and H2O and metabolism of hydrogen peroxide have been measured in leaves of alfalfa (Medicago sativa L. cv.. Aragón) under drought stress. The inhibitory effect of drought upon photosynthesis depended on the severity of the stress treatment. Leaf water potential (Ψleaf) down to -28 MPa reduced CO2 availability due to! stomata! closure and inhibited the rate of photosynthesis.. Leaf water potential lower than -2.8 MPa directly affected CO2 fixation, although CO2 was not limiting. Transpiration was more affected by stomata! closure than photosynthesis, which led to an apparent improvement in WUE (water use efficiency). Alfalfa leaves with Ψleaf lower than -.2.0 MPa had an increased quantum requirement, probably due to the severe stress. effect on photoenergetic reactions. Ethylene evolution from alfalfa leaves increased when they were subjected to Ψleaf of -1.6 MPa. Under more severe stress, the leaves showed low or almost no ethylene production. In parallel with the increase in ethylene production, alfalfa leaves exhibited an increased membrane lipid peroxidation index (malondialdehyd.e content) and an increased peroxide content. Superoxide .disniutase activity (SOD; EC 1.15.1.1) was not affected by drought stress. Catalase (EC 1.11.1.6) was inhibited at slight stress but significantly increased at a Ψleaf of -2.0 MPa. Peroxidase (EC 1.11.1.7) was progressively inhibited as drought stress developed. The possible implication of reactive O2 intermediates in drought stress-induced senescence of alfalfa leaves is discussed in the light of the pattern of enzymatic scavenging systems. [ABSTRACT FROM AUTHOR]

The effect of drought upon phosphoenolpyruvate carboxylase (PEPC; EC 4.1.1.31), malate dehydrogenase (MDH; EC 1.1.1.37), alcohol dehydrogenase (ADH; EC 1.1.1.1) and β-hydroxybutyrate dehydrogenase (β-OH-BDH; EC 1.1.130) enzyme activities as well as the leghemoglobin (Lb), malate and ethanol contents of alfalfa nodules (Medicago sativa L. cv Aragón) were examined. Both the leghemoglobin (Lb) content and the Lb/soluble protein ratio were significantly reduced at a nodule water potential (ψmod ) of -1.3 MPa. At lower ψmod,Lb content decreased further, but the ratio remained unchanged. Slight stress (-1.3 MPa) drastically affected acetylene reduction activity (ARA; 60% reduction)' whereas in vitro PEPC activity was maintained at relatively constant values. As stress progressed (-2.0 MPa), a simultaneous reduction in both activities was observed. Severe stress (ψmodlower than: -2.0 MPa) stimulated in vitro PEPC. Bacteroid β-OH-BDFI activity was stimulated by slight (-1.3 MPa) and moderate (-2.0 MPa) drought. MDH activity rose in slightly stressed nodules (ψmod -1.3 MPa). Greater water deficits sharply decreased. MDH activity to! values significant]y lower than those found in control nodules. Nodule malate content followed the same pattern as MDH. The plant fraction of the nodule showed constitutive ADH activity and contained ethanol, ADH was stimulated at slight (-1.3 MPa) and moderate drought levels (-2.0 MPa). Ethanol content showed similar behavior to ADH activity. Inhibition of ARA, reduction of Lb con tent and stimulation of the fermentative metabolism induced by water stress suggest some reduction in O2 availability within the nodule. [ABSTRACT FROM AUTHOR]

Nerve growth factor (NGF) is a potential therapeutic agent for Alzheimer's disease (AD) as it has positive effects on the basal forebrain cholinergic neurons whose degeneration correlates with the cognitive decline in AD. We have previously described an encapsulated cell biodelivery device, NsG0202, capable of local delivery of NGF by a genetically modified human cell line, NGC-0295. The NsG0202 devices have shown promising safety and therapeutic results in a small phase 1b clinical study. However, results also show that the NGF dose could advantageously be increased. We have used the sleeping beauty transposon expression technology to establish a new clinical grade cell line, NGC0211, with at least 10 times higher NGF production than that of NGC-0295. To test whether encapsulation of this cell line provides a relevant dose escalation step in delivering NGF for treatment of the cognitive decline in AD patients, we have validated the bioactivity of devices with NGC0211 and NGC-0295 cells in normal rat striatum as well as in the quinolinic acid striatal lesion model. These preclinical animal studies show that implantation of devices with NGC0211 cells lead to significantly higher NGF output, which in both cases correlate with highly improved potency. [ABSTRACT FROM AUTHOR]

The article discusses the acetylene reduction in relaxation to levels of phosphate and fixed nitrogen in Green Bay. This paper reports data collected during the summer of 1971 in the southern part of Green Bay of Lake Michigan. This study undertook to characterize acetylene reduction and nutrient levels throughout the summer. Water samples were collected. Phytoplankton characteristically increase rapidly in the spring. A high soluble phosphate concentration in the water preceded all major increases in the population of heterocystous, nitrogen-fixing blue-green algae.

The immunoprotective nature of the testis has led to numerous investigations for its ability to protect cellular grafts. Sertoli cells (SCs) are at least partially responsible for this immunoprotective environment and survive allogeneic and xenogeneic transplantation. The ability of SCs to survive transplantation leads to the possibility that they could be engineered to deliver therapeutic proteins. As a model to test this hypothesis, we examined the ability of SCs that produce green fluorescent protein (GFP) to survive transplantation and continue expressing GFP. SCs were isolated from transgenic mice engineered to express GFP and transplanted as aggregates under the kidney capsule of severe combined immunodeficient (SCID) and Balb/c mice. Using this paradigm, it was possible to compare the survival of transgenic SCs directly in both immunodeficient and immunocompetent recipients. Fluorescence microscopy of the kidney capsule and immunohistochemistry of the grafts for GFP and GATA-4 revealed the presence of GFP-expressing SCs under the kidney capsule of SCID and Balb/c mice at both 30 and 60 days post-transplantation. In contrast, islets transplanted to Balb/c mice were rejected. Thus, SCs survive transplantation and continue to express GFP raising the possibility that SCs can be engineered using transgenic technology to produce proteins, such as insulin, factor VIII, or dopamine for the treatment of diabetes, hemophilia or Parkinson's disease, respectively.Gene Therapy (2004) 11, 694-700. doi:10.1038/sj.gt.3302218 Published online 15 January 2004 [ABSTRACT FROM AUTHOR]

The genes encoding malate synthase (glcB) and isocitrate lyase (aceA) and a 240-bp open reading frame (SMc00767) located downstream of aceA were isolated and functionally characterized in Sinorhizobium meliloti. Independent and double interposon mutants of each gene were constructed, and the corresponding phenotypes were analyzed. aceA mutants failed to grow on acetate, and mutants deficient in SMc00767 were also affected in acetate utilization. In contrast, mutants deficient in glcB grew on acetate similar to wild-type strain Rm5000. Complementation experiments showed that aceA and SMc00767 gene constructs were able to restore the growth on acetate in the corresponding single mutants. aceA-glcB, aceA-SMc00767, and glcB-SMc00767 double knockouts were also unable to grow on acetate, but this ability was recovered when the wild-type aceA-glcB or aceA-SMc00767 loci were introduced into the double mutants. These data confirm the functional role of aceA and SMc00767 and show that glcB, in the absence of SMc00767, is required for acetate metabolism. Isocitrate lyase and malate synthase activities were measured in strain Rm5000, the mutant derivatives, and complemented strains. aceA and glcB were able to complement the enzymatic activity lacking in the corresponding single mutants. The enzymatic activities also showed that SMc00767 represses the activity of isocitrate lyase in cells grown on acetate. Gene fusions confirmed the repressor role of SMc00767, which regulates aceA expression at the transcriptional level. Comparison of the transcriptional profiles of the SMc00767 mutant and wild-type strain Rm5000 showed that SMc00767 represses the expression of a moderate number of open reading frames, including aceA; thus, we propose that SMc00767 is a novel repressor involved in acetate metabolism in S. meliloti. Genetic and functional analyses indicated that aceA and SMc00767 constitute a functional two-gene operon, which is conserved in other α-proteobacteria. Alfalfa plants infected with the aceA and glcB mutants were not impaired in nodulation or nitrogen fixation, and so the glyoxylate cycle is not required in the Rhizobium-legume symbiosis. [ABSTRACT FROM AUTHOR]