Botulinum toxin: treatment of self-mutilation in patients with Lesch-Nyhan syndrome.

Lesch-Nyhan syndrome (LNS) is a rare X-linked recessive disorder involving purine metabolism caused by the congenital absence of hypoxanthine guanine phosphoribosyl transferase. A characteristic feature of LNS is the appearance of intractable self-injurious behavior, usually in the form of severe lip and finger biting. The mechanism behind this severe self-mutilating behavior is unknown, and is one of the main challenges in the management of this condition.

Understanding and predicting ecological dynamics: are major surprises inevitable?

Ecological surprises, substantial and unanticipated changes in the abundance of one or more species that result from previously unsuspected processes, are a common outcome of both experiments and observations in community and population ecology. Here, we give examples of such surprises along with the results of a survey of well-established field ecologists, most of whom have encountered one or more surprises over the course of their careers. Truly surprising results are common enough to require their consideration in any reasonable effort to characterize nature and manage natural resources.

Coral reef habitats as surrogates of species, ecological functions, and ecosystem services.

Habitat maps are often the core spatially consistent data set on which marine reserve networks are designed, but their efficacy as surrogates for species richness and applicability to other conservation measures is poorly understood. Combining an analysis of field survey data, literature review, and expert assessment by a multidisciplinary working group, we examined the degree to which Caribbean coastal habitats provide useful planning information on 4 conservation measures: species richness, the ecological functions of fish species, ecosystem processes, and ecosystem services. Approximately one-quarter to one-third of benthic invertebrate species and fish species (disaggregated by life phase; hereafter fish species) occurred in a single habitat, and Montastraea-dominated forereefs consistently had the highest richness of all species, processes, and services.

Protein extraction for proteome analysis from cacao leaves and meristems, organs infected by Moniliophthora perniciosa, the causal agent of the witches' broom disease.

Preparation of high-quality proteins from cacao vegetative organs is difficult due to very high endogenous levels of polysaccharides and polyphenols. In order to establish a routine procedure for the application of proteomic and biochemical analysis to cacao tissues, three new protocols were developed; one for apoplastic washing fluid (AWF) extraction, and two for protein extraction--under denaturing and nondenaturing conditions. The first described method allows a quick and easy collection of AWF--using infiltration-centrifugation procedure--that is representative of its composition in intact leaves according to the smaller symplastic contamination detected by the use of the hexose phosphate isomerase marker.

Neuroacanthocytosis and carbamazepine responsive paroxysmal dyskinesias.

Neuroacanthocytosis (NA) comprises a genetically heterogeneous group of disorders characterized by dysfunction of the erythrocyte membrane, presenting with acanthocytes and several neurological manifestations. We report the case of a 42-year-old woman with NA who in addition had episodes consistent with paroxysmal dyskinesias. She was diagnosed with NA featuring mild chorea, weakness of the right lower extremity and myoclonic jerks.

Use of response surface methodology to examine chitinase regulation in the basidiomycete Moniliophthora perniciosa.

We report here the first analysis of chitinase regulation in Moniliophthora perniciosa, the causal agent of the witches' broom disease of cacao. A multivariate statistical approach was employed to evaluate the effect of several variables, including carbon and nitrogen sources and cultivation time, on M. perniciosa non-secreted (detected in mycelium, i.

Phenylethynyl-pyrrolo[1,2-a]pyrazine: a new potent and selective tool in the mGluR5 antagonists arena.

The synthesis and the structure activity of a new series of pyrrolo[1,2-a]pyrazine is reported. These molecules are potent and selective non-competitive mGluR5 antagonists and may shed new light on the pattern of substitution tolerated by this receptor.

A global map of human impact on marine ecosystems.

The management and conservation of the world's oceans require synthesis of spatial data on the distribution and intensity of human activities and the overlap of their impacts on marine ecosystems. We developed an ecosystem-specific, multiscale spatial model to synthesize 17 global data sets of anthropogenic drivers of ecological change for 20 marine ecosystems. Our analysis indicates that no area is unaffected by human influence and that a large fraction (41%) is strongly affected by multiple drivers.

New fused benzazepine as selective D3 receptor antagonists. Synthesis and biological evaluation. Part one: [h]-fused tricyclic systems.

The synthesis and SAR of a new series of potent and selective dopamine D(3) receptor antagonists is reported. The introduction of a tricyclic [h]-fused benzazepine moiety on the recently disclosed scaffold of 1,2,4-triazol-3-yl-thiopropyl-tetrahydrobenzazepines is reported. A full rat pharmacokinetic characterization is also reported.

Selective dopamine D3 receptor antagonists: a review 2001-2005.

A growing body of evidence indicates that dopamine (DA) D(3) receptors are significantly involved in the control of drug-seeking behavior, and may play an important role in the pathophysiology of impulse control disorders and schizophrenia. This hypothesis has been difficult to test due to the lack of compounds with high selectivity for central DA D(3) receptors. Recently, however, the synthesis and characterization of new highly potent and selective DA D(3) receptor antagonists has permitted to characterize the role of the DA D(3) receptor in a wide range of preclinical animal models.

New fused benzazepine as selective D3 receptor antagonists. Synthesis and biological evaluation. Part 2: [g]-fused and hetero-fused systems.

The synthesis and the SAR of a new series of potent and selective dopamine D(3) receptor antagonists is reported. The new scaffolds of the [g]-fused and the hetero-fused tricyclic benzazepine are here reported together with their pharmacokinetic profile.

Pectin methyl esterase inhibits intrusive and symplastic cell growth in developing wood cells of Populus.

Wood cells, unlike most other cells in plants, grow by a unique combination of intrusive and symplastic growth. Fibers grow in diameter by diffuse symplastic growth, but they elongate solely by intrusive apical growth penetrating the pectin-rich middle lamella that cements neighboring cells together. In contrast, vessel elements grow in diameter by a combination of intrusive and symplastic growth.

Pallidal surgery for the treatment of primary generalized dystonia: long-term follow-up.

Objective: To describe the results and long-term follow-up after functional surgery of the internal segment of the globus pallidus (GPi) in 10 patients with primary generalized dystonia.

Patients And Methods: Nine of the 10 patients were positive for the DYT1 gene mutation. Bilateral deep brain stimulation (DBS) of the GPi was performed in three cases, bilateral pallidotomy in two, and combined surgery (unilateral GPi lesion with contralateral stimulation) in the remaining five.

Evaluating and ranking the vulnerability of global marine ecosystems to anthropogenic threats.

Marine ecosystems are threatened by a suite of anthropogenic stressors. Mitigating multiple threats is a daunting task, particularly when funding constraints limit the number of threats that can be addressed. Threats are typically assessed and prioritized via expert opinion workshops that often leave no record of the rationale for decisions, making it difficult to update recommendations with new information.

1,2,4-triazol-3-yl-thiopropyl-tetrahydrobenzazepines: a series of potent and selective dopamine D(3) receptor antagonists.

The discovery of new highly potent and selective dopamine D3 receptor antagonists has recently permitted characterization of the role of the dopamine D3 receptor in a wide range of preclinical animal models. A novel series of 1,2,4-triazol-3-yl-thiopropyl-tetrahydrobenzazepines demonstrating a high level of D3 affinity and selectivity with an excellent pharmacokinetic profile is reported here. In particular, the pyrazolyl derivative 35 showed good oral bioavailability and brain penetration associated with high potency and selectivity in vitro.

Posterior spinal cord stimulation in a case of painful legs and moving toes.

A 59-year-old woman with a 5-year history of right lower limb pain is reported. Symptoms developed initially when walking and progressively became bilateral, appeared at rest and involuntary movements of the toes became evident. A diagnosis of painful legs and moving toes was made.

Human impacts on the species-area relationship in reef fish assemblages.

The relationship between species richness and area is one of the oldest, most recognized patterns in ecology. Here we provide empirical evidence for strong impacts of fisheries exploitation on the slope of the species-area relationship (SAR). Using comparative field surveys of fish on protected and exploited reefs in three oceans and the Mediterranean Sea, we show that exploitation consistently depresses the slope of the SAR for both power-law and exponential models.

Comparative analysis of expressed genes from cacao meristems infected by Moniliophthora perniciosa.

Background And Aims: Witches' broom disease is caused by the hemibiotrophic basidiomycete Moniliophthora perniciosa, and is one of the most important diseases of cacao in the western hemisphere. Because very little is known about the global process of such disease development, expressed sequence tags (ESTs) were used to identify genes expressed during the Theobroma cacao-Moniliophthora perniciosa interaction.

Methods: Two cDNA libraries corresponding to the resistant (RT) and susceptible (SP) cacao-M.

Characterization of necrosis and ethylene-inducing proteins (NEP) in the basidiomycete Moniliophthora perniciosa, the causal agent of witches' broom in Theobroma cacao.

The hemibiotrophic basidiomycete Moniliophthora perniciosa causes witches' broom disease of Theobroma cacao. Analysis of the M. perniciosa draft genome led to the identification of three putative genes encoding necrosis and ethylene-inducing proteins (MpNEPs), which are apparently located on the same chromosome.

Trophic cascade facilitates coral recruitment in a marine reserve.

Reduced fishing pressure and weak predator-prey interactions within marine reserves can create trophic cascades that increase the number of grazing fishes and reduce the coverage of macroalgae on coral reefs. Here, we show that the impacts of reserves extend beyond trophic cascades and enhance the process of coral recruitment. Increased fish grazing, primarily driven by reduced fishing, was strongly negatively correlated with macroalgal cover and resulted in a 2-fold increase in the density of coral recruits within a Bahamian reef system.

Holmes tremor secondary to brainstem hemorrhage responsive to levodopa: report of 2 cases.

We report 2 patients who presented a brainstem hemorrhage and who, after 1 and 6 months, respectively, developed a 4-Hz postural and resting tremor consistent with Holmes tremor, which severely interfered with the activities of daily living. In both cases, levodopa dramatically improved the tremor. Pharmacological treatment of this condition is usually disappointing, and surgical procedures are commonly required for severe cases.

Identification of new potential regulators of the Medicago truncatula-Sinorhizobium meliloti symbiosis using a large-scale suppression subtractive hybridization approach.

We set up a large-scale suppression subtractive hybridization (SSH) approach to identify Medicago truncatula genes differentially expressed at different stages of the symbiotic interaction with Sinorhizobium meliloti, with a particular interest for regulatory genes. We constructed 7 SSH libraries covering successive stages from Nod factor signal transduction to S. meliloti infection, nodule organogenesis, and functioning.