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ABSTRACT The limited temporal completeness and taxonomic accuracy of species lists, made available in a traditional manner in scientific publications, has always represented a problem. These lists are invariably limited to a few taxonomic groups and do not represent up-to-date knowledge of all species and classifications. In this context, the Brazilian megadiverse fauna is no exception, and the Catálogo Taxonômico da Fauna do Brasil (CTFB) (http://fauna.jbrj.gov.br/), made public in 2015, represents a database on biodiversity anchored on a list of valid and expertly recognized scientific names of animals in Brazil. The CTFB is updated in near real time by a team of more than 800 specialists. By January 1, 2024, the CTFB compiled 133,691 nominal species, with 125,138 that were considered valid. Most of the valid species were arthropods (82.3%, with more than 102,000 species) and chordates (7.69%, with over 11,000 species). These taxa were followed by a cluster composed of Mollusca (3,567 species), Platyhelminthes (2,292 species), Annelida (1,833 species), and Nematoda (1,447 species). All remaining groups had less than 1,000 species reported in Brazil, with Cnidaria (831 species), Porifera (628 species), Rotifera (606 species), and Bryozoa (520 species) representing those with more than 500 species. Analysis of the CTFB database can facilitate and direct efforts towards the discovery of new species in Brazil, but it is also fundamental in providing the best available list of valid nominal species to users, including those in science, health, conservation efforts, and any initiative involving animals. The importance of the CTFB is evidenced by the elevated number of citations in the scientific literature in diverse areas of biology, law, anthropology, education, forensic science, and veterinary science, among others.

ABSTRACT The tribe Scatellini comprises 247 species (plus five nomina dubia) that are distributed in all biogeographical regions except Antarctica. The tribe currently includes nine genera. One genus, Scatella Robineau-Desvoidy, 1830, includes six subgenera. To test the monophyly of Scatellini and to understand the phylogenetic relationships among the included genera, a cladistic analysis was performed. The optimization criterion chosen was parsimony using implicit character weighting, and the analysis was based on the morphological characters of adult males and females. The species of eight of nine genera of Scatellini from different geographic regions were studied. Species of the other tribes of Ephydrinae were also included to provide insight on the phylogenetic position of Scatellini within this subfamily. The implied weighting analysis showed that the tribe Scatellini, as currently characterized, is a monophyletic group, as are all the genera included in it. From this analysis, two major lineages emerged: 1. Scatella and its included subgenera; 2. All other genera of Scatellini. The latter clade includes (Thinoscatella (Lamproscatella + Haloscatella)) and the clade (Amalopteryx (Philotelma (Limnellia + Scatophila))). Five subgenera of Scatella were recovered as monophyletic groups: Parascatella Cresson, Synhoplos Lamb, Apulvillus Malloch, Scatella, and Teichomyza Macquart. Neoscatella Malloch is synonymized with Scatella sensu stricto. In the implied weighting analysis, Ephydrini and Scatellini are sister-groups. Based on this phylogenetic reconstruction, the taxonomy of Scatellini is presented at the generic level. A key to the included genera and subgenera is also presented.

The systematic and somewhat controversial history of Risa Becker is presented, and its relationship with Diasemocera Bezzi (tribe Psilopini, Ephydridae) is documented by morphological evidence and an association with host plants in the family Amaranthaceae. The tribe Risini Papp (as Risidae) is synonymized with Psilopini Cresson. Notorisa gen. nov., from Australia, is described (type species: Notorisa mcalpinei sp. nov.; Australia. Victoria: Big Desert National Park, near Lake Hindmarsh; 36°03.7'S 141°54.8'E). Achaetorisa Papp is retained as a subgenus within Risa and includes five species, including two new combinations: Risa brevicornis (Papp) comb. nov., Risa salsolae (Mathis & Zatwarnicki) comb. nov., and two new species: R. brevirostris sp. nov. (Israel. Ẕomet Zohar; 31°08.5'N 35°21.6'E) and R. nettae sp. nov. (Israel. Ẕomet Zohar; 31°08.5'N 35°21.6'E). A fourth new species is described in the subgenus Risa: R. (Risa) kotrbae sp. nov. (Israel. Ẕomet Zohar; 31°08.5'N 35°21.6'E).

Heterozygous de novo loss-of-function mutations in the gene expression regulator HNRNPU cause an early-onset developmental and epileptic encephalopathy. To gain insight into pathological mechanisms and lay the potential groundwork for developing targeted therapies, we characterized the neurophysiologic and cell-type-specific transcriptomic consequences of a mouse model of HNRNPU haploinsufficiency. Heterozygous mutants demonstrated global developmental delay, impaired ultrasonic vocalizations, cognitive dysfunction and increased seizure susceptibility, thus modeling aspects of the human disease. Single-cell RNA-sequencing of hippocampal and neocortical cells revealed widespread, yet modest, dysregulation of gene expression across mutant neuronal subtypes. We observed an increased burden of differentially-expressed genes in mutant excitatory neurons of the subiculum-a region of the hippocampus implicated in temporal lobe epilepsy. Evaluation of transcriptomic signature reversal as a therapeutic strategy highlights the potential importance of generating cell-type-specific signatures. Overall, this work provides insight into HNRNPU-mediated disease mechanisms and provides a framework for using single-cell RNA-sequencing to study transcriptional regulators implicated in disease.

De novo mutations in GNB1, encoding the Gβ1 subunit of G proteins, cause a neurodevelopmental disorder with global developmental delay and epilepsy, GNB1 encephalopathy. Here, we show that mice carrying a pathogenic mutation, K78R, recapitulate aspects of the disorder, including developmental delay and generalized seizures. Cultured mutant cortical neurons also display aberrant bursting activity on multi-electrode arrays. Strikingly, the antiepileptic drug ethosuximide (ETX) restores normal neuronal network behavior in vitro and suppresses spike-and-wave discharges (SWD) in vivo. ETX is a known blocker of T-type voltage-gated Ca2+ channels and G protein-coupled potassium (GIRK) channels. Accordingly, we present evidence that K78R results in a gain-of-function (GoF) effect by increasing the activation of GIRK channels in cultured neurons and a heterologous model (Xenopus oocytes)—an effect we show can be potently inhibited by ETX. This work implicates a GoF mechanism for GIRK channels in epilepsy, identifies a new mechanism of action for ETX in preventing seizures, and establishes this mouse model as a pre-clinical tool for translational research with predicative value for GNB1 encephalopathy.

The black twig borer, Xylosandrus compactus (Eichhoff), is one of the few ambrosia beetles that infests healthy plants. In Florida, orchids (Dendrobium spp. and Cattleya spp.), and avocado (Persea americana Mill.) have sustained notable economic losses (Dekle and Kuitert 1968, McClanahan 1951). Although ornamental shrubs and shade trees seldom die from beetle infestations, the loss of growth and aesthetics can be substantial. Elsewhere, the black twig borer is a serious pest of coffee, Coffea canephora Pierre (esp. var. robusta Ineac) (Brader 1964). This document is EENY-311, one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: December 2003. Revised: September 2005. EENY311/IN577: Black Twig Borer, Xylosandrus compactus (Eichhoff) (Insecta: Coleoptera: Curculionidae: Scolytinae (ufl.edu)

The Nantucket pine tip moth, Rhyacionia frustrana (Comstock), is a serious pest of young pine in plantations, wild pine seedlings in open areas, Christmas tree plantings, ornamental pines, and pine seed orchards in the United States. Growth loss and stem deformity, caused by larvae feeding inside growing shoots, buds, and conelets, can be considerable during the first five years when most damage occurs (Yates et al. 1981). The increasing population of a preferred host species, loblolly pine (Pinus taeda L.), in Florida poses an ever-increasing problem of Nantucket pine tip moth infestations. This document is EENY-304 (originally published as DPI Entomology Circular 298), one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: August 2003. EENY304/IN581: Nantucket Pine Tip Moth, Rhyacionia frustrana (Comstock) (Insecta: Lepidoptera: Tortricidae) (ufl.edu)

Summary: Absence seizures, manifested by spike-wave discharges (SWD) in the electroencephalogram, display synchronous reciprocal excitation between the neocortex and thalamus. Recent studies have revealed that inhibitory neurons in the reticular thalamic (RT) nucleus and excitatory thalamocortical (TC) neurons are two subcortical players in generating SWD. However, the signals that drive SWD-related activity remain elusive. Here, we show that SWD predominately occurs during wakefulness in several mouse models of absence epilepsy. In more focused studies of Gnb1 mutant mice, we found that sensory input regulates SWD. Using in vivo recording, we demonstrate that TC cells are activated prior to the onset of SWD and then inhibited during SWD. On the contrary, RT cells are slightly inhibited prior to SWD, but are strongly activated during SWD. Furthermore, chemogenetic activation of TC cells leads to the enhancement of SWD. Together, our results indicate that sensory input can regulate SWD by activating the thalamocortical pathway.

Abstracts: Background: Aseptic loosening of total joint replacements (TJRs) continues to be the main cause of implant failures. The socioeconomic impact of surgical revisions is hugely significant; in the United Kingdom alone, it is estimated that £137 m is spent annually on revision arthroplasties. Enhancing the longevity of titanium implants will help reduce the incidence and overall cost of failed devices. Methods: In realising the development of a superior titanium technology, we exploited the natural affinity of titanium for phosphonic acids and developed a facile means of coating the metal with (3S)1-fluoro-3-hydroxy-4-(oleoyloxy)butyl-1-phosphonate (FHBP), a phosphatase-resistant analogue of lysophosphatidic acid (LPA). Importantly LPA and selected LPA analogues like FHBP synergistically cooperate with calcitriol to promote human osteoblast formation and maturation. Results: Herein, we provide evidence that simply immersing titanium in aqueous solutions of FHBP afforded a surface that was superior to unmodified metal at enhancing osteoblast maturation. Importantly, FHBP-functionalised titanium remained stable to 2 years of ambient storage, resisted ∼35 kGy of gamma irradiation and survived implantation into a bone substitute (Sawbone™) and irrigation. Conclusion: The facile step we have taken to modify titanium and the robustness of the final surface finish are appealing properties that are likely to attract the attention of implant manufacturers in the future. The translational potential of this article: We have generated a functionalised titanium (Ti) surface by simply immersing Ti in aqueous solutions of a bioactive lipid. As a facile procedure it will have greater appeal to implant manufacturers compared to onerous and costly developmental processes.

Paucity of the glucose transporter-1 (Glut1) protein resulting from haploinsufficiency of the SLC2A1 gene arrests cerebral angiogenesis and disrupts brain function to cause Glut1 deficiency syndrome (Glut1 DS). Restoring Glut1 to Glut1 DS model mice prevents disease, but the precise cellular sites of action of the transporter, its temporal requirements, and the mechanisms linking scarcity of the protein to brain cell dysfunction remain poorly understood. Here, we show that Glut1 functions in a cell-autonomous manner in the cerebral microvasculature to affect endothelial tip cells and, thus, brain angiogenesis. Moreover, brain endothelial cell–specific Glut1 depletion not only triggers a severe neuroinflammatory response in the Glut1 DS brain, but also reduces levels of brain-derived neurotrophic factor (BDNF) and causes overt disease. Reduced BDNF correlated with fewer neurons in the Glut1 DS brain. Controlled depletion of the protein demonstrated that brain pathology and disease severity was greatest when Glut1 scarcity was induced neonatally, during brain angiogenesis. Reducing Glut1 at later stages had mild or little effect. Our results suggest that targeting brain endothelial cells during early development is important to ensure proper brain angiogenesis, prevent neuroinflammation, maintain BDNF levels, and preserve neuron numbers. This requirement will be essential for any disease-modifying therapeutic strategy for Glut1 DS.

Mouse models have made innumerable contributions to understanding the genetic basis of neurological disease and pathogenic mechanisms and to therapy development. Here we consider the current state of mouse genetic models of Developmental and Epileptic Encephalopathy (DEE), representing a set of rare but devastating and largely intractable childhood epilepsies. By examining the range of mouse lines available in this rapidly moving field and by detailing both expected and unusual features in representative examples, we highlight lessons learned in an effort to maximize the full potential of this powerful resource for preclinical studies.

Mutations in the X-linked gene IQSEC2 are associated with multiple cases of epilepsy, epileptic encephalopathy, intellectual disability and autism spectrum disorder, the mechanistic understanding and successful treatment of which remain a significant challenge in IQSEC2 and related neurodevelopmental genetic diseases. To investigate disease etiology, we studied behaviors and synaptic function in IQSEC2 deficient mice. Hemizygous Iqsec2 null males exhibit growth deficits, hyperambulation and hyperanxiety phenotypes. Adult hemizygotes experience lethal spontaneous seizures, but paradoxically have a significantly increased threshold to electrically induced limbic seizures and relative resistance to chemically induced seizures. Although there are no gross defects in brain morphology, hemizygotes exhibit stark hippocampal reactive astrogliosis. Electrophysiological recordings of hippocampal neurons reveal increased excitatory drive specifically onto interneurons, and significant alterations in intrinsic electrical properties specific to the interneuron population. As they age, hemizygotes also develop an increased abundance of parvalbumin-positive interneurons in the hippocampus, neurons in which IQSEC2 is expressed in addition to the excitatory neurons. These findings point to a novel role of IQSEC2 in hippocampal interneuron synaptic function and development with implications for a class of intractable neurodevelopmental diseases.

ARFGEF1 encodes a guanine exchange factor involved in intracellular vesicle trafficking, and is a candidate gene for childhood genetic epilepsies. To model ARFGEF1 haploinsufficiency observed in a recent Lennox Gastaut Syndrome patient, we studied a frameshift mutation (Arfgef1fs) in mice. Arfgef1fs/+ pups exhibit signs of developmental delay, and Arfgef1fs/+ adults have a significantly decreased threshold to induced seizures but do not experience spontaneous seizures. Histologically, the Arfgef1fs/+ brain exhibits a disruption in the apical lining of the dentate gyrus and altered spine morphology of deep layer neurons. In primary hippocampal neuron culture, dendritic surface and synaptic but not total GABAA receptors (GABAAR) are reduced in Arfgef1fs/+ neurons with an accompanying decrease in the number of GABAAR-containing recycling endosomes in cell body. Arfgef1fs/+ neurons also display differences in the relative ratio of Arf6+:Rab11+:TrfR+ recycling endosomes. Although the GABAAR-containing early endosomes in Arfgef1fs/+ neurons are comparable to wildtype, Arfgef1fs/+ neurons show an increase in the number of GABAAR-containing lysosomes in dendrite and cell body. Together, the altered endosome composition and decreased neuronal surface GABAAR results suggests a mechanism whereby impaired neuronal inhibition leads to seizure susceptibility.

Abstract Background This paper describes the means by which a United States National Institute on Drug Abuse (NIDA)-funded cooperative, Juvenile Justice-Translational Research on Interventions for Adolescents in the Legal System (JJ-TRIALS), utilized an established implementation science framework in conducting a multi-site, multi-research center implementation intervention initiative. The initiative aimed to bolster the ability of juvenile justice agencies to address unmet client needs related to substance use while enhancing inter-organizational relationships between juvenile justice and local behavioral health partners. Methods The EPIS (Exploration, Preparation, Implementation, Sustainment) framework was selected and utilized as the guiding model from inception through project completion; including the mapping of implementation strategies to EPIS stages, articulation of research questions, and selection, content, and timing of measurement protocols. Among other key developments, the project led to a reconceptualization of its governing implementation science framework into cyclical form as the EPIS Wheel. The EPIS Wheel is more consistent with rapid-cycle testing principles and permits researchers to track both progressive and recursive movement through EPIS. Moreover, because this randomized controlled trial was predicated on a bundled strategy method, JJ-TRIALS was designed to rigorously test progress through the EPIS stages as promoted by facilitation of data-driven decision making principles. The project extended EPIS by (1) elucidating the role and nature of recursive activity in promoting change (yielding the circular EPIS Wheel), (2) by expanding the applicability of the EPIS framework beyond a single evidence-based practice (EBP) to address varying process improvement efforts (representing varying EBPs), and (3) by disentangling outcome measures of progression through EPIS stages from the a priori established study timeline. Discussion The utilization of EPIS in JJ-TRIALS provides a model for practical and applied use of implementation frameworks in real-world settings that span outer service system and inner organizational contexts in improving care for vulnerable populations. Trial registration NCT02672150. Retrospectively registered on 22 January 2016.

RNA-protein interaction plays important roles in post-transcriptional regulation. Recent advancements in cross-linking and immunoprecipitation followed by sequencing (CLIP-seq) technologies make it possible to detect the binding peaks of a given RNA binding protein (RBP) at transcriptome scale. However, it is still challenging to predict the functional consequences of RBP binding peaks. In this study, we propose the Protein-RNA Association Strength (PRAS), which integrates the intensities and positions of the binding peaks of RBPs for functional mRNA targets prediction. We illustrate the superiority of PRAS over existing approaches on predicting the functional targets of two related but divergent CELF (CUGBP, ELAV-like factor) RBPs in mouse brain and muscle. We also demonstrate the potential of PRAS for wide adoption by applying it to the enhanced CLIP-seq (eCLIP) datasets of 37 RNA decay related RBPs in two human cell lines. PRAS can be utilized to investigate any RBPs with available CLIP-seq peaks. PRAS is freely available at http://ouyanglab.jax.org/pras/.

Dynamin 1 is a large neuron-specific GTPase involved in the endocytosis and recycling of pre-synaptic membranes and synaptic vesicles. Mutations in the gene encoding dynamin 1 (DNM1) underlie two epileptic encephalopathy syndromes, Lennox-Gastaut Syndrome and Infantile Spasms. Mice homozygous for the Dnm1 “fitful” mutation, a non-synonymous coding variant in an alternatively spliced exon of Dnm1 (exon 10a; isoform designation: Dnm1aFtfl) have an epileptic encephalopathy-like disorder including lethal early onset seizures, locomotor and neurosensory deficits. Although fitful heterozygotes have milder recurrent seizures later in life, suggesting an additive or semi-dominant mechanism, the molecular etiology must also consider the fact that Dnm1aFtfl exerts a dominant negative effect on endocytosis in vitro. Another complication is that the fitful mutation induces alterations in the relative abundance of Dnm1 splice variants; mutants have a downregulation of Dnm1a and an upregulation of Dnm1b, changes which may contribute to the epileptic pathology. To examine whether Dnm1a loss of function, Dnm1aFtfl dominance or compensation by Dnm1b is the most critical for severe seizures, we studied alternate isoform-specific mutant mice. Mice lacking Dnm1 exon 10a or Dnm1 exon 10b have neither spontaneous seizures nor other overt abnormalities, suggesting that in normal conditions the major role of each isoform is redundant. However, in the presence of Dnm1aFtfl only exon 10a deleted mice experience severe seizures. These results reveal functional differences between Dnm1a and Dnm1b isoforms in the presence of a challenge, i.e. toxic Dnm1Ftfl, while reinforcing its effect explicitly in this model of severe pediatric epilepsy.

As plurilingual/multilingual research advances, factors that contribute to or hinder individual's flexible language use are still underexplored. Questions such as "Why do some people identify as plurilingual and pluricultural while others do not?" and "What factors contribute to high levels of plurilingual and pluricultural competence (PPC)?" remain unknown. This article presents results of a mixed methods study with 250 plurilingual participants in the multilingual city of Montreal, Canada. Data was collected through a demographic questionnaire, the Plurilingual and Pluricultural Identity Questionnaire (PPIQ) and the Plurilingual and Pluricultural Competence (PPC) scale. Results show that a vast majority of participants identified as plurilingual and pluricultural, and seven reasons for shaping this identity were found: rich repertoire, lived experiences, awareness of differences and similarities, transculturalism, adaptation and integration, translanguaging, and openness. Moreover, there was a significant difference in PPC scores between Canadian-born participants and participants born outside of Canada, suggesting that speakers of minority languages in Canada have lower PPC scores compared to speakers of Canada's official languages. These results are significant as they enhance our understanding of language use and identity, suggesting that plurilingualism and pluriculturalism can be the new norm in multilingual contexts but minority languages need further recognition.

Plurilingualism is an inclusive language teaching approach to sustain multilingual societies, but there is little investigation on teacher candidates' (TCs) beliefs and challenges before and after its implementation. This interpretive qualitative study introduced plurilingualism in teacher education at a Canadian university. Sixteen TCs participated in the study, which 1) investigated TCs' conceptualizations of plurilingualism, and 2) examined TCs' perceptions of overall affordances of critical plurilingual pedagogies before and after their practicum. For four months, participants experimented with plurilingual pedagogies such as translanguaging and cross-linguistic analysis, designed tasks, and taught lessons. Five types of data were generated: 1) weekly annotations of readings on Perusall, 2) designed language tasks, 3) task delivery demonstrations, 4) lesson plans, and 5) final reflection after the teaching practicum. Inductive content analysis was conducted on NVivo with data triangulation. Findings suggest that TCs shifted their views of language, and aligned plurilingual pedagogies with equity, diversity, inclusion and decoloniality principles. Findings also show that TCs transgressed the monolingual discourses often present in schools, and felt empowered after the training. We call for the inclusion of critical plurilingual practices in teacher education programs for the sustainability of multi/plurilingualism.

Here we present an open-source R package 'meaRtools' that provides a platform for analyzing neuronal networks recorded on Microelectrode Arrays (MEAs). Cultured neuronal networks monitored with MEAs are now being widely used to characterize in vitro models of neurological disorders and to evaluate pharmaceutical compounds. meaRtools provides core algorithms for MEA spike train analysis, feature extraction, statistical analysis and plotting of multiple MEA recordings with multiple genotypes and treatments. meaRtools functionality covers novel solutions for spike train analysis, including algorithms to assess electrode cross-correlation using the spike train tiling coefficient (STTC), mutual information, synchronized bursts and entropy within cultured wells. Also integrated is a solution to account for bursts variability originating from mixed-cell neuronal cultures. The package provides a statistical platform built specifically for MEA data that can combine multiple MEA recordings and compare extracted features between different genetic models or treatments. We demonstrate the utilization of meaRtools to successfully identify epilepsy-like phenotypes in neuronal networks from Celf4 knockout mice. The package is freely available under the GPL license (GPL> = 3) and is updated frequently on the CRAN web-server repository. The package, along with full documentation can be downloaded from: https://cran.r-project.org/web/packages/meaRtools/.

Scatellini was proposed by Wirth & Stone, 1956 and currently includes 242 described species in nine genera. The tribe has representatives in all biogeographic regions and about 70 species occur in the Neotropical Region. Prior to this study, only two species were recorded from Brazil: Scatella obscura and Limnellia itatiaia. In this paper, species of Scatellini from Brazil are reviewed with an emphasis on the fauna from southern Brazil, where six new species have been discovered and are described herein: Scatella praia, S. plaumanni, S. rara, Scatophila darrowae, S. dianneae, and S. prainha. To facilitate identification of the tribe and included genera and species, we have included diagnoses of these taxa and have also provided an annotated key to the Neotropical genera in Scatellini. We have also provided illustrations of structures of the male terminalia of all included species of the genus Scatophila.

Canadian multilingualism and multiculturalism are on the rise. Yet, monolingual language instruction remains the standard: students are often discouraged from using their additional languages and teaching materials still lack a plurilingual lens. To further inform the practice of plurilingual pedagogies, this paper reports on results of a convergent mixed methods study that investigated the plurilingual learning strategies of 20 adult English as an additional language (EAL) student tutors and tutees in a Francophone college in Montréal. The study asked (1) What plurilingual strategies do EAL tutors and tutees use to teach and learn English from each other? (2) What are their perceptions of the affordances and challenges of these plurilingual strategies? Data from an observation grid, fieldnotes, and semi-structured interviews were analyzed deductively and inductively, and merged for convergence analysis. Results show that (1) participants regularly engaged in plurilingual practices including translation, translanguaging, and crosslinguistic comparisons during the tutoring sessions. Further, (2) participants perceived plurilingual strategies as useful for supporting English language development, fostering positive learning experience and conceptual links; however, they noted challenges pertaining to the monolingual posture of EAL instruction, to English oral production, and to the feasibility of plurilingual pedagogies. Implications for EAL education in multilingual contexts like Canada are discussed.

Species of the shore-fly Parydra Stenhammar, 1844 from Brazil are reviewed with an emphasis on the fauna from southern Brazil, where one new species, Parydra arcuata sp. nov. - state of Paraná, municipality of Castro (8 km N, 24º45.3'S, 49º58.9'W) -, has been discovered and is herein described. To facilitate identification of species, we have included a diagnosis of the genus. We have also provided illustrations of structures of the male terminalia for all included species.

Mutations in the neuronal voltage-gated sodium channel genes SCN1A and SCN2A are associated with inherited epilepsies, including genetic epilepsy with febrile seizures plus (GEFS+) and Dravet syndrome (severe myoclonic epilepsy of infancy). The clinical presentation and severity of these epilepsies vary widely, even in people with the same mutation, suggesting the action of environmental or genetic modifiers. To gain support for the hypothesis that genetic modifiers can influence clinical presentation in patients with SCN1A-derived GEFS+, we used mouse models to study the effect of combining the human GEFS+ mutation SCN1A-R1648H with SCN2A, KCNQ2, and SCN8A mutations. Knock-in mice heterozygous for the R1648H mutation (Scn1aRH/+) have decreased thresholds to induced seizures and infrequent spontaneous seizures, whereas homozygotes display spontaneous seizures and premature lethality. Scn2aQ54 transgenic mice have a mutation in Scn2a that results in spontaneous, adult-onset partial motor seizures, and mice carrying the Kcnq2-V182M mutation exhibit increased susceptibility to induced seizures, and rare spontaneous seizures as adults. Combining the Scn1a-R1648H allele with either Scn2aQ54 or Kcnq2V182M/+ results in early-onset, generalized tonic–clonic seizures and juvenile lethality in double heterozygous mice. In contrast, Scn8a mutants exhibit increased resistance to induced seizures. Combining the Scn1a-R1648H and Scn8a-med-jo alleles restores normal thresholds to flurothyl-induced seizures in Scn1aRH/+ heterozygotes and improved survival of Scn1aRH/RH homozygotes. Our results demonstrate that variants in Scn2a, Kcnq2, and Scn8a can dramatically influence the phenotype of mice carrying the Scn1a-R1648H mutation and suggest that ion channel variants may contribute to the clinical variation seen in patients with monogenic epilepsy.

The species of Diphuia Cresson, 1944 are reviewed with an emphasis on the fauna from southern Brazil, where two new species have been discovered and herein are described: Diphuia antonina sp. nov. and Diphuia grandis sp. nov. All known species are placed into two species groups (the anomala and nitida groups), which are characterized, and a key to these species is included. To facilitate identification of species of this uncommon genus, we have included diagnoses of the genus and tribe Hecamedini and have also provided an annotated key to New World genera in the tribe and to the known species of Diphuia. We have also provided illustrations of structures of the male terminalia of all included species.The species from southern Brazil, including the new ones, are illustrated.

The childhood epileptic encephalopathies (EE's) are seizure disorders that broadly impact development including cognitive, sensory and motor progress with severe consequences and comorbidities. Recently, mutations in DNM1 (dynamin 1) have been implicated in two EE syndromes, Lennox-Gastaut Syndrome and Infantile Spasms. Dnm1 encodes dynamin 1, a large multimeric GTPase necessary for activity-dependent membrane recycling in neurons, including synaptic vesicle endocytosis. Dnm1Ftfl or "fitful" mice carry a spontaneous mutation in the mouse ortholog of DNM1 and recapitulate many of the disease features associated with human DNM1 patients, providing a relevant disease model of human EE's. In order to examine the cellular etiology of seizures and behavioral and neurological comorbidities, we engineered a conditional Dnm1Ftfl mouse model of DNM1 EE. Observations of Dnm1Ftfl/flox mice in combination with various neuronal subpopulation specific cre strains demonstrate unique seizure phenotypes and clear separation of major neurobehavioral comorbidities from severe seizures associated with the germline model. This demonstration of pleiotropy suggests that treating seizures per se may not prevent severe comorbidity observed in EE associated with dynamin-1 mutations, and is likely to have implications for other genetic forms of EE.

Alzheimer's disease (AD) is a leading cause of dementia in the elderly and is characterized by amyloid plaques, neurofibrillary tangles (NFTs) and neuronal dysfunction. Early onset AD (EOAD) is commonly caused by mutations in amyloid precursor protein (APP) or genes involved in the processing of APP including the presenilins (e.g. PSEN1 or PSEN2). In general, mouse models relevant to EOAD recapitulate amyloidosis, show only limited amounts of NFTs and neuronal cell dysfunction and low but significant levels of seizure susceptibility. To investigate the effect of genetic background on these phenotypes, we generated APPswe and PSEN1de9 transgenic mice on the seizure prone inbred strain background, DBA/2J. Previous studies show that the DBA/2J genetic background modifies plaque deposition in the presence of mutant APP but the impact of PSEN1de9 has not been tested. Our study shows that DBA/2J.APPswePSEN1de9 mice are significantly more prone to premature lethality, likely to due to lethal seizures, compared to B6.APPswePSEN1de9 mice-70% of DBA/2J.APPswePSEN1de9 mice die between 2-3 months of age. Of the DBA/2J.APPswePSEN1de9 mice that survived to 6 months of age, plaque deposition was greatly reduced compared to age-matched B6.APPswePSEN1de9 mice. The reduction in plaque deposition appears to be independent of microglia numbers, reactive astrocytosis and complement C5 activity.

Diopsosoma Malloch, 1932 and its only included species, D. primum Malloch, 1932 are redescribed. While the genus is remarkable and easily recognized by its laterally elongate head that is produced into eyestalks, it is otherwise virtually unknown. To bring attention to this extraordinary fly we redescribe it within the context of brief descriptions of the family Periscelididae and subfamily Periscelidinae. We also provide an annotated key to the described genera of Periscelididae and a photographic montage of the holotype, which is perhaps the only preserved specimen of this genus and species.Redescrição do gênero Diopsosoma Malloch (Diptera, Periscelididae). Diopsosoma Malloch, 1932 e sua única espécie conhecida, D. primum Malloch, 1932 são redescritos. O gênero, embora facilmente reconhecível pela cabeça projetada lateralmente em pedúnculos oculares, é praticamente desconhecido. Por isso, apresenta-se sua redescrição em conjunto com breves redescrições de Periscelididae e da subfamília Periscelidinae. Chave para os gêneros de Periscelididae e fotomontagem do holótipo, talvez o único exemplar preservado da espécie, são fornecidas.

Absence epilepsy (AE) is a common type of genetic generalized epilepsy (GGE), particularly in children. AE and GGE are complex genetic diseases with few causal variants identified to date. Gria4 deficient mice provide a model of AE, one for which the common laboratory inbred strain C3H/HeJ (HeJ) harbors a natural IAP retrotransposon insertion in Gria4 that reduces its expression 8-fold. Between C3H and non-seizing strains such as C57BL/6, genetic modifiers alter disease severity. Even C3H substrains have surprising variation in the duration and incidence of spike-wave discharges (SWD), the characteristic electroencephalographic feature of absence seizures. Here we discovered extensive IAP retrotransposition in the C3H substrain, and identified a HeJ-private IAP in the Pcnxl2 gene, which encodes a putative multi-transmembrane protein of unknown function, resulting in decreased expression. By creating new Pcnxl2 frameshift alleles using TALEN mutagenesis, we show that Pcnxl2 deficiency is responsible for mitigating the seizure phenotype - making Pcnxl2 the first known modifier gene for absence seizures in any species. This finding gave us a handle on genetic complexity between strains, directing us to use another C3H substrain to map additional modifiers including validation of a Chr 15 locus that profoundly affects the severity of SWD episodes. Together these new findings expand our knowledge of how natural variation modulates seizures, and highlights the feasibility of characterizing and validating modifiers in mouse strains and substrains in the post-genome sequence era.

Anacamptodes pergracilis (Hulst), commonly known as the cypress looper, drew considerable attention in late summer of 1980 with the unexpected defoliation of nearly 28,000 ha of cypress trees in USFS-NPS Big Cypress National Preserve (Collier and Monroe counties). Currently, cypress looper populations are at low levels, even in the Fisheating Creek (Glades County) area, a perennial generator of significant cypress looper defoliation over the past 20 years. This 3-page fact sheet was written by Wayne N. Dixon and published by the UF Department of Entomology and Nematology, April 2013. http://edis.ifas.ufl.edu/in986

RNA-binding proteins have emerged as causal agents of complex neurological diseases. Mice deficient for neuronal RNA-binding protein CELF4 have a complex neurological disorder with epilepsy as a prominent feature. Human CELF4 has recently been associated with clinical features similar to those seen in mutant mice. CELF4 is expressed primarily in excitatory neurons, including large pyramidal cells of the cerebral cortex and hippocampus, and it regulates excitatory but not inhibitory neurotransmission. We examined mechanisms underlying neuronal hyperexcitability in Celf4 mutants by identifying CELF4 target mRNAs and assessing their fate in the absence of CELF4 in view of their known functions. CELF4 binds to at least 15%-20% of the transcriptome, with striking specificity for the mRNA 3' untranslated region. CELF4 mRNA targets encode a variety of proteins, many of which are well established in neuron development and function. While the overall abundance of these mRNA targets is often dysregulated in Celf4 deficient mice, the actual expression changes are modest at the steady-state level. In contrast, by examining the transcriptome of polysome fractions and the mRNA distribution along the neuronal cell body-neuropil axis, we found that CELF4 is critical for maintaining mRNA stability and availability for translation. Among biological processes associated with CELF4 targets that accumulate in neuropil of mutants, regulation of synaptic plasticity and transmission are the most prominent. Together with a related study of the impact of CELF4 loss on sodium channel Na(v)1.6 function, we suggest that CELF4 deficiency leads to abnormal neuronal function by combining a specific effect on neuronal excitation with a general impairment of synaptic transmission. These results also expand our understanding of the vital roles RNA-binding proteins play in regulating and shaping the activity of neural circuits.

Revised! EENY-321, a 6-page illustrated fact sheet by Michael C. Thomas, Wayne N. Dixon, and Thomas R. Fasulo, is part of the Featured Creatures collection. It describes this serious pest of pines in Europe that is considered a potential threat to some of the pine species cultivated in Florida — distribution, identification, biology, economic importance, survey, and management. Includes references. Published by the UF Department of Entomology and Nematology, September 2010. EENY-321/IN596: Pine Shoot Beetle, Tomicus piniperda (Linnaeus) (Insecta: Coleoptera: Curculionidae: Scolytinae) (ufl.edu)

Dynamin-1 (Dnm1) encodes a large multimeric GTPase necessary for activity-dependent membrane recycling in neurons, including synaptic vesicle endocytosis. Mice heterozygous for a novel spontaneous Dnm1 mutation--fitful--experience recurrent seizures, and homozygotes have more debilitating, often lethal seizures in addition to severe ataxia and neurosensory deficits. Fitful is a missense mutation in an exon that defines the DNM1a isoform, leaving intact the alternatively spliced exon that encodes DNM1b. The expression of the corresponding alternate transcripts is developmentally regulated, with DNM1b expression highest during early neuronal development and DNM1a expression increasing postnatally with synaptic maturation. Mutant DNM1a does not efficiently self-assemble into higher order complexes known to be necessary for proper dynamin function, and it also interferes with endocytic recycling in cell culture. In mice, the mutation results in defective synaptic transmission characterized by a slower recovery from depression after trains of stimulation. The DNM1a and DNM1b isoform pair is highly conserved in vertebrate evolution, whereas invertebrates have only one isoform. We speculate that the emergence of more specialized forms of DNM1 may be important in organisms with complex neuronal function.

Revised! EENY-245, a 5-page illustrated fact sheet by Wayne N. Dixon and T. R. Fasulo, is part of the Featured Creatures collection. It describes this insect that attacks over half of the cultivated plant species grown in the United States, including increasing feeding damage on conifer seedlings — distribution, description, life history, hosts, damage, survey and detection, and management. Includes references. Published by the UF Department of Entomology and Nematology, November 2009. EENY-245/IN513: Tarnished Plant Bug, Lygus lineolaris (Palisot de Beauvois) (Insecta: Hemiptera: Miridae) (ufl.edu)

Idiopathic epilepsy is a common human disorder with a strong genetic component, usually exhibiting complex inheritance. We describe a new mouse mutation in C57BL/6J mice, called frequent-flyer (Ff), in which disruption of the gene encoding RNA-binding protein Bruno-like 4 (Brunol4) leads to limbic and severe tonic-clonic seizures in heterozygous mutants beginning in their third month. Younger heterozygous adults have a reduced seizure threshold. Although homozygotes do not survive well on the C57BL/6J background, on mixed backgrounds homozygotes and some heterozygotes also display spike-wave discharges, the electroencephalographic manifestation of absence epilepsy. Brunol4 is widely expressed in the brain with enrichment in the hippocampus. Gene expression profiling and subsequent analysis revealed the down-regulation of at least four RNA molecules encoding proteins known to be involved in neuroexcitability, particularly in mutant hippocampus. Genetic and phenotypic assessment suggests that Brunol4 deficiency in mice results in a complex seizure phenotype, likely due to the coordinate dysregulation of several molecules, providing a unique new animal model of epilepsy that mimics the complex genetic architecture of common disease.

EENY-368, a 5-page illustrated fact sheet by Wayne N. Dixon, describes the distribution, identification, biology, economic importance, symptoms, and management of this family of oak tree pests. Includes bibliographic references. This publication has been published as DPI Entomology Circular 355 (Nov. 1992) and is also available on the Featured Creatures Web site by the UF Department of Entomology and Nematology, May 2006. EENY-368/IN664: Gall Wasps, Callirhytis quercusclaviger (Ashmead) and Callirhytis cornigera (Osten Sacken) (Insecta: Hymenoptera: Cynipidae) (ufl.edu)

Tomicus piniperda (Linnaeus), a pine shoot beetle native to Europe, was first discovered in the United States in July 1992, in a Christmas tree plantation in Ohio. Since then, intensive surveying by the U.S. Department of Agriculture and state departments of agriculture has revealed its presence (as of 29 December 2003) in twelve northern states: Illinois, Indiana, Maine, Maryland, Michigan, New Hampshire, New York, Ohio, Pennsylvania, Vermont, West Virginia and Wisconsin (Anonymous 2004b). Because T. piniperda occurs about as far south in the Old World as the latitude of Florida, it is considered a potential threat to at least some of the pine species intensively cultivated in Florida. This document is EENY-321 (originally published as DPI Entomology Circular 354), one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: April 2004. Revised: September 2005. EENY-321/IN596: Pine Shoot Beetle, Tomicus piniperda (Linnaeus) (Insecta: Coleoptera: Curculionidae: Scolytinae) (ufl.edu)

Pine sawfly larvae, Neodiprion spp., are the most common defoliating insects of pine trees, Pinus spp., in Florida. Sawfly infestations can cause growth loss and mortality, especially when followed by secondary attack by bark and wood-boring beetles (Coleoptera: Buprestidae, Cerambycidae, Scolytidae,). Trees of all ages are susceptible to sawfly defoliation (Barnard and Dixon 1983, Coppel and Benjamin 1965). This document is EENY-317 (originally published as DPI Entomology Circular No. 258), one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: January 2004. EENY317/IN592: Pine Sawflies, Neodiprion spp. (Insecta: Hymenoptera: Diprionidae) (ufl.edu)

Naupactus leucoloma Boheman, the whitefringed beetle, was first collected in North America near Svea, Florida in 1936 (Buchanan 1939). This species and two others (N. minor (Buchanan) and N. peregrinus (Buchanan)) comprise the whitefringed beetle complex in North America (Buchanan 1947, Warner 1975). Whitefringed beetles are considered serious pests of many agricultural crops (Young et al. 1950) and have become pests of young pines planted on converted croplands in the South. This document is EENY-294 (originally published as DPI Entomology Circular 309), one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: June 2003. EENY294/IN572: Whitefringed Beetles, Naupactus (=Graphognathus) spp. (Insecta: Coleoptera: Curculionidae) (ufl.edu)

Lygus lineolaris (Palisot de Beauvois), the tarnished plant bug, attacks a wide variety of economically important herbaceous plants, vegetable crops, commercial flower plants, fruit trees, and nursery stock (Kelton 1975). In fact, over half of the cultivated plant species grown in the United States are listed as host plants for tarnished plant bugs (Capinera 2001). This document is EENY-245 (originally published as DPI Entomology Circular 320), one of a series of Featured Creatures from the Entomology and Nematology Department, Florida Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: November 2001. EENY-245/IN513: Tarnished Plant Bug, Lygus lineolaris (Palisot de Beauvois) (Insecta: Hemiptera: Miridae) (ufl.edu)

The southern pine beetle (SPB), Dendroctonus frontalis Zimmermann, is the most destructive insect pest of pine in the southern United States. A recent historical review estimated that SPB caused $900 million of damage to pine forests from 1960 through 1990 (Price et a1. 1992). This aggressive tree killer is a native insect that lives predominantly in the inner bark of pine trees. Trees attacked by SPB often exhibit hundreds of resin masses (i.e., pitch tubes) on the outer tree bark. SPB feed on phloem tissue where they construct winding S-shaped or serpentine galleries. The galleries created by both the adult beetles and their offspring can effectively girdle a tree, causing its death. SPB also carry, and introduce into trees, blue-stain fungi. These fungi colonize xylem tissue and block water flow within the tree, also causing tree mortality (Thatcher and Conner 1985). Consequently, once SPB have successfully colonized a tree, the tree cannot survive, regardless of control measures. his document is EENY-176 (originally published as DPI Entomology Circular 369), one of the Featured Creatures series of the Entomology and Nematology Department, Cooperative Extension Service, Institute of Food and Agricultural Sciences, University of Florida. Published: November 2000. Revised: March 2004. EENY-176/IN333: Southern Pine Beetle, Dendroctonus frontalis (Coleoptera: Curculionidae: Scolytinae) (ufl.edu)

Although interorganizational relationships (IORs) are essential to the effective delivery of human services, very little research has examined relationships between juvenile justice agencies and behavioral health providers, and few studies have identified the most critical organizational and individual-level characteristics influencing IORs. Across 36 sites, juvenile probation officials (n = 458) and community behavioral health providers (n = 91) were surveyed about characteristics of their agencies, themselves, and IORs with each other. Generalized Linear Mixed Models were used to analyze the data. The strongest predictors included Perceived Organizational Support and individual Adaptability. Implications for research, theory and practice are discussed.