Clinical, neuroradiological, and molecular characterization of mitochondrial threonyl-tRNA-synthetase (TARS2)-related disorder.

Purpose: Biallelic variants in TARS2, encoding the mitochondrial threonyl-tRNA-synthetase, have been reported in a small group of individuals displaying a neurodevelopmental phenotype but with limited neuroradiological data and insufficient evidence for causality of the variants.

Methods: Exome or genome sequencing was carried out in 15 families. Clinical and neuroradiological evaluation was performed for all affected individuals, including review of 10 previously reported individuals.

Imaging the distribution of iron oxide nanoparticles in hypothermic perfused tissues.

Purpose: Herein, we evaluate the use of MRI as a tool for assessing iron oxide nanoparticle (IONP) distribution within IONP perfused organs and vascularized composite allografts (VCAs) (i.e., hindlimbs) prepared for cryopreservation.

Economic Evaluation of the Juvenile Drug Court/Reclaiming Futures (JDC/RF) Model.

Juvenile drug court (JDC) programs are an increasingly popular option for rehabilitating juvenile offenders with substance problems, but research has found inconsistent evidence regarding their effectiveness and economic impact. While assessing client outcomes such as reduced substance use and delinquency is necessary to gauge program effectiveness, a more comprehensive understanding of program success and sustainability can be attained by examining program costs and economic benefits. As part of the National Cross-Site Evaluation of JDC and Reclaiming Futures (RF), an economic analysis of five JDC/RF programs was conducted from a multisystem and multiagency perspective.

Efficacy of Two Systemic Insecticides With Stem Gall Wasp, Zapatella davisae (Hymenoptera: Cynipidae) on Black Oak.

Black oak, Quercus velutina Lamarck, is the dominant deciduous tree on Cape Cod, Nantucket, and Martha's Vineyard, Massachusetts, and in recent years it has experienced widespread mortality and severe canopy loss due to infestations of a stem gall wasp, Zapatella davisae Buffington and Melika (Hymenoptera: Cynipidae). A single application of systemic insecticides emamectin benzoate and imidacloprid was found to reduce or prevent further accumulation of Z. davisae damage on infested black oak during a 1-yr trial.

Perceived barriers and difficulties of intermittent catheterization: In Korean patients with spinal dysraphism and their parents.

Aims: Integrating regular intermittent catheterization (IC) into daily life is essential for good medical outcomes in patients with neurogenic bladders. The goal is to identify long-term IC-related barriers, or difficulties in Korean patients with spinal dysraphism and their parents.

Methods: The data were prospectively collected using questionnaires from spinal dysraphism patients from two sources: an online community, and those visiting the outpatient clinic of Seoul National university hospital.

Imbalanced expression of Vcan mRNA splice form proteins alters heart morphology and cellular protein profiles.

The fundamental importance of the proteoglycan versican to early heart formation was clearly demonstrated by the Vcan null mouse called heart defect (hdf). Total absence of the Vcan gene halts heart development at a stage prior to the heart's pulmonary/aortic outlet segment growth. This creates a problem for determining the significance of versican's expression in the forming valve precursors and vascular wall of the pulmonary and aortic roots.

The Oct1 homolog Nubbin is a repressor of NF-κB-dependent immune gene expression that increases the tolerance to gut microbiota.

Background: Innate immune responses are evolutionarily conserved processes that provide crucial protection against invading organisms. Gene activation by potent NF-κB transcription factors is essential both in mammals and Drosophila during infection and stress challenges. If not strictly controlled, this potent defense system can activate autoimmune and inflammatory stress reactions, with deleterious consequences for the organism.

Wild-type Drosophila melanogaster as a model host to analyze nitrogen source dependent virulence of Candida albicans.

The fungal pathogen Candida albicans is a common cause of opportunistic infections in humans. We report that wild-type Drosophila melanogaster (OrR) flies are susceptible to virulent C. albicans infections and have established experimental conditions that enable OrR flies to serve as model hosts for studying C.

Basic leucine zipper protein Cnc-C is a substrate and transcriptional regulator of the Drosophila 26S proteasome.

While the 26S proteasome is a key proteolytic complex, little is known about how proteasome levels are maintained in higher eukaryotic cells. Here we describe an RNA interference (RNAi) screen of Drosophila melanogaster that was used to identify transcription factors that may play a role in maintaining levels of the 26S proteasome. We used an RNAi library against 993 Drosophila transcription factor genes to identify genes whose suppression in Schneider 2 cells stabilized a ubiquitin-green fluorescent protein reporter protein.

The POU transcription factor Drifter/Ventral veinless regulates expression of Drosophila immune defense genes.

Innate immunity operates as a first line of defense in multicellular organisms against infections caused by different classes of microorganisms. Antimicrobial peptides (AMPs) are synthesized constitutively in barrier epithelia to protect against microbial attack and are also upregulated in response to infection. Here, we implicate Drifter/Ventral veinless (Dfr/Vvl), a class III POU domain transcription factor, in tissue-specific regulation of the innate immune defense of Drosophila.

Psychometric properties of an assessment for mental health recovery programs.

The concept of recovery can be operationalized from either the point of view of the consumer, or from the perspective of the agency providing services. The Milestones of Recovery Scale (MORS) was created to capture aspects of recovery from the agency perspective. Evidence establishing the psychometric properties of the MORS was obtained in three efforts: Inter-rater reliability using staff at The Village, a multi-service organization serving the homeless mentally ill in Long Beach, California; inter-rater reliability was also obtained from Vinfen Corporation, a large provider of housing services to mentally ill persons in Boston, Massachusetts.

Patterns of recovery from severe mental illness: a pilot study of outcomes.

We performed a pilot study examining the patterns of recovery from severe mental illness in a model integrated service delivery system using measures from the Milestones of Recovery Scale (MORS), a valid and reliable measure of recovery outcomes which ranges from 1 to 8 (8 levels). For purposes of presentation, we constructed an aggregate MORS (6 levels) where the levels are described as follows: (1) extreme risk; (2) unengaged, poorly self-coordinating; (3) engaged, poorly self-coordinating; (4) coping and rehabilitating; (5) early recovery, and (6) self reliant. We analyzed MORS data on individuals followed over time from The Village in Long Beach, California (658 observations).

Expressed breast milk: safety in the hospital.

The Pennsylvania Patient Safety Reporting System (PA-PSRS, pronounced PAY-sirs) is a confidential, statewide reporting system on the Internet to which all Pennsylvania hospitals, outpatient-surgery facilities, and birthing centers, as well as some abortion facilities, were required to file information on medical errors beginning in June 2004.Safety Monitor, this column in AJN from PA-PSRS, informs nurses on issues that can affect patient safety and presents strategies they can integrate easily into practice.For more information on PA-PSRS, visit the Web site of Pennsylvania's Patient Safety Authority, at www.

Monte carlo model of stricture formation in photodynamic therapy of normal pig esophagus.

Photodynamic therapy (PDT) is FDA-approved for use in patients with Barrett's esophagus using porfimer sodium (2 mg per kg) and a recommended light dose of 130 J cm(-1) for high grade dysplasia. Despite uniform drug and light doses, the clinical outcome of PDT is variable. A significant number of PDT cases result in esophageal strictures, a side effect related to excessive energy absorption.

A member of the p38 mitogen-activated protein kinase family is responsible for transcriptional induction of Dopa decarboxylase in the epidermis of Drosophila melanogaster during the innate immune response.

Drosophila innate immunity is controlled primarily by the activation of IMD (immune deficiency) or Toll signaling leading to the production of antimicrobial peptides (AMPs). IMD signaling also activates the JUN N-terminal kinase (JNK) cascade, which is responsible for immune induction of non-antimicrobial peptide immune gene transcription though the transcription factor AP-1. Transcription of the Dopa decarboxylase (Ddc) gene is induced in response to gram-negative and gram-positive septic injury, but not aseptic wounding.

Attention deficit hyperactivity disorder: parents' and professionals' perceptions.

Attention deficit hyperactivity disorder (ADHD) is a prevalent childhood psychiatric condition. This study was a qualitative investigation with parents and professionals conducted in two north London boroughs, using focus groups as well as semi-structured and narrative interviews. The aim was to explore parents' and professionals' beliefs regarding the causes of ADHD and their perceptions of service provision.

Novel events associated with phenotypic reversion of a P element mutant in Drosophila melanogaster.

Transposable P elements have been used extensively for Drosophila mutagenesis. While their mutagenic activity has long been recognized, the mechanisms by which P elements cause mutations are varied and not completely understood. We describe here an experiment to replace a P element at vestigial (vg) that caused a strong mutant phenotype (P[21-3]) with a P element (P[21]) known to produce a very weak phenotype when inserted at vg.