Reelin signaling is impaired in autism.

Background: Autism is a severe neurodevelopmental disorder with genetic and environmental etiologies. Recent genetic linkage studies implicate Reelin glycoprotein in causation of autism. To further investigate these studies, brain levels of Reelin protein and mRNA and mRNAs for VLDLR, Dab-1, and GSK3 were investigated.

Avascular necrosis of the talus: a pictorial essay.

The talus is predisposed to avascular necrosis (AVN), or bone death due to ischemia, owing to its unique structure, characteristic extraosseous arterial sources, and variable intraosseous blood supply. Both traumatic and atraumatic causes have been implicated in talar AVN. The risk of posttraumatic AVN can be predicted using the Hawkins classification system.

Melting characteristics of fat present on the surface of industrial spray-dried dairy powders.

The melting characteristics of the fat present on the surface (surface free-fat) of two industrial spray-dried dairy powders (cream powder and whole milk powder) were investigated in comparison with those of other milk fat fractions present in the powder, such as free-fat from the interior of the powder particle (inner free-fat) and encapsulated fat. The melting characteristics of the milk fat fractions were studied by fatty acid composition, melting profile and solid fat content profile. The results indicated that all milk fat fractions including surface free-fat contained various triglycerides with melting points ranging from -40 to +40 degrees C.

Tazarotene cream (0.1%) in combination with betamethasone valerate foam (0.12%) for plaque-type psoriasis.

A combination of multiple agents is often required to achieve treatment success for plaque-type psoriasis. We report a case series of 10 patients that were treated with betamethasone valerate foam (0.12%) in the morning and topical tazarotene cream (0.

Regulation of GluR1 abundance in murine hippocampal neurones by serum- and glucocorticoid-inducible kinase 3.

Phosphatidylinositol 3 kinase (PI3-kinase) is activated during and is required for hippocampal glutamate receptor-dependent long-term potentiation. It mediates the delivery of AMPA receptors to the neuronal surface. Among the downstream targets of PI3-kinase are three members of the serum- and glucocorticoid-inducible kinase family, SGK1, SGK2 and SGK3.

SGK1: a rapid aldosterone-induced regulator of renal sodium reabsorption.

Recently, substantial progress has been made in understanding the mechanisms by which aldosterone rapidly stimulates sodium transport in the distal nephron and other tight epithelia. Serum- and glucocorticoid-regulated kinase 1 (SGK1) has been identified as an important mediator of this process. Its physiological relevance has been revealed through heterologous expression in cultured cells and generation of SGK1 knockout mice.

CD4 and chemokine receptors on human brain microvascular endothelial cells, implications for human immunodeficiency virus type 1 pathogenesis.

Central nervous system (CNS) dysfunction is commonly observed in children with human immunodeficiency virus type 1 (HIV-1) infection, but the mechanism(s) whereby HIV-1 causes encephalopathy remains incompletely understood. Human brain microvascular endothelial cells (HBMECs), which constitute the blood-brain barrier, are likely to contribute to HIV-1 encephalopathy, but it is unclear whether HIV-1 receptors (CD4, chemokine receptors) are present on HBMECs. In the present study, the presence of CD4 in six different children was demonstrated.

Class I topical corticosteroid use by psoriasis patients in an academic practice.

Background: Psoriasis is a chronic skin disorder that can have a profound impact on the quality of life of patients. The treatment of psoriasis is complicated by the availability of numerous topical agents, systemic agents, and phototherapy. Of the topical preparations available, the ultra-high potency, or Class I steroids, have an important role in treating psoriasis.

Autoimmunity to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.

The pathogenic mechanisms underlying Batten disease are unclear. Patients uniformly possess autoantibodies against glutamic acid decarboxylase (GAD) that are predominantly reactive with a region of GAD (amino acids 1 to 20) distinct from subjects with autoimmune type 1 diabetes or stiff-person syndrome. Batten patients did not possess autoantibodies against other type 1 diabetes-associated autoantigens and human leukocyte antigen genotypes revealed no specific associations with this disease.

Quality of stroke care within a hospital: effects of a mobile stroke service.

Objective: An Australian stroke services study (SCOPES) has developed a framework to compare different forms of acute stroke services, the gold standard being localised stroke units. We aimed to use this framework to assess changes in the quality of stroke care over time as a sequential audit process.

Design And Setting: A retrospective medical record audit comparing 100 sequential stroke admissions (July 2002 to June 2003) two years after institution of a mobile stroke service (MSS) with 100 historical controls (September 1998 to October 1999) at a 260-bed hospital in Melbourne.

Interaction among Btn1p, Btn2p, and Ist2p reveals potential interplay among the vacuole, amino acid levels, and ion homeostasis in the yeast Saccharomyces cerevisiae.

Btn2p, a novel cytosolic coiled-coil protein in Saccharomyces cerevisiae, was previously shown to interact with and to be necessary for the correct localization of Rhb1p, a regulator of arginine uptake, and Yif1p, a Golgi protein. We now report the biochemical and physical interactions of Btn2p with Ist2p, a plasma membrane protein that is thought to have a function in salt tolerance. A deletion in Btn2p (btn2Delta strains) results in a failure to correctly localize Ist2p, and strains lacking Btn2p and Ist2p (btn2Delta ist2Delta strains) are unable to grow in the presence of 0.

CLN3, the protein associated with batten disease: structure, function and localization.

Batten disease, an inherited neurodegenerative storage disease affecting children, results from the autosomal recessive inheritance of mutations in Cln3. The function of the CLN3 protein remains unknown. A key to understanding the pathology of this devastating disease will be to elucidate the function of CLN3 at the cellular level.

Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease.

The neuronal ceroid lipofuscinoses (NCLs, also known collectively as Batten disease) are a group of lysosomal storage disorders characterized by the accumulation of autofluorescent storage material in the brain and other tissues. A number of genes underlying various forms of NCL have been cloned, but the basis for the neurodegeneration in any of these is unknown. High levels of dolichol pyrophosphoryl oligosaccharides have previously been demonstrated in brain tissue from several NCL patients, but the specificity of the effect for the NCLs has been unclear.

Isolation and properties of methanesulfonate-degrading Afipia felis from Antarctica and comparison with other strains of A. felis.

Three novel strains of methylotrophic Afipia felis were isolated from several locations on Signy Island, Antarctica, and a fourth from estuary sediment from the River Douro, Portugal. They were identified as strains of the alpha-2 proteobacterium A. felis by 16S rRNA gene sequence analysis.

Medication-related factors affecting health care outcomes and costs for patients with psoriasis in the United States.

Background: The impact of psoriasis medication therapy on costs and patient outcomes in large nationally representative samples needs further examination.

Objective: This study examined the association between factors related to medication use, health status, and health care costs associated with psoriasis in the United States.

Methods: A cross-sectional cohort study was performed using the 2000 Medical Expenditure Panel Survey database.

The cost of psoriasis therapies: considerations for therapy selection.

Purpose: The purpose of this article is to provide a review of the cost of psoriasis therapies from two sources as well as compare the average wholesale price (AWP), as listed in the 2003 Drug Topics Red Book to that of a popular Internet pharmacy.

Methods: Prices of therapies were obtained two ways: the AWP was recorded from the 2003 Drug Topics Red Book. A range and average price per gram (or mL) were calculated based on the smallest size or quantity available.

Enhanced mitochondrial degradation of yeast cytochrome c with amphipathic structures.

The dispensable N-terminus of iso-1-cytochrome c (iso-1) in the yeast Saccharomyces cerevisiae was replaced by 11 different amphipathic structures. Rapid degradation of the corresponding iso-1 occurred, with the degree of degradation increasing with the amphipathic moments; and this amphipathic-dependent degradation was designated ADD. ADD occurred with the holo-forms in the mitochondria but not as the apo-forms in the cytosol.

Glutamic acid decarboxylase autoimmunity in Batten disease and other disorders.

Degenerative diseases of the CNS, such as stiff-person syndrome (SPS), progressive cerebellar ataxia, and Rasmussen encephalitis, have been characterized by the presence of autoantibodies. Recent findings in individuals with Batten disease and in animal models for the disorder indicate that this condition may be associated with autoantibodies against glutamic acid decarboxylase (GAD), an enzyme that converts the excitatory neurotransmitter glutamate to the inhibitory neurotransmitter gamma-aminobutyric acid (GABA). Anti-GAD autoantibodies could result in excess excitatory neurotransmitters, leading to the seizures and other symptoms observed in patients with Batten disease.

Steroid receptor coactivator-1 splice variants differentially affect corticosteroid receptor signaling.

The mechanisms of receptor- and cell-specific effects of the adrenal corticosteroid hormones via mineralo- (MRs) and glucocorticoid receptors (GRs) are still poorly understood. Because the expression levels of two splice variants of the steroid receptor coactivator-1 (SRC-1) 1a and 1e, can differ significantly in certain cell populations, we tested the hypothesis that their relative abundance could determine cell- and receptor-specific effects of corticosteroid receptor-mediated transcription. In transient transfections, we demonstrate three novel types of SRC-1a- and SRC-1e-specific effects for corticosteroid receptors.

A preliminary study of airborne microbial biodiversity over Peninsular Antarctica.

This study used PCR-based molecular biological identification techniques to examine the biodiversity of air sampled over Rothera Point (Antarctic Peninsula). 16S rDNA fragments of 132 clones were sequenced and identified to reveal a range of microorganisms, including cyanobacteria, actinomycetes, diatom plastids and other uncultivated bacterial groups. Matches for microorganisms that would be considered evidence of human contamination were not found.

A Simplified Psoriasis Area Severity Index (SPASI) for rating psoriasis severity in clinic patients.

Introduction: The Psoriasis Area and Severity Index (PASI) is the most widely used tool to assess psoriasis disease severity in clinical trials, although it can be exceedingly cumbersome for use in daily clinical practice. Because clinical trials rely on the PASI for inclusion criteria, having a PASI score on a clinic patient may be useful for determining if the patient has a level of disease severity similar to that of patients treated in clinical trials.

Purpose: The purpose of this study is to assess a simplified measure of psoriasis disease severity that is more conducive to use in general dermatology practice, the simplified PASI (SPASI).

Construction and evaluation of genetically engineered replication-defective porcine reproductive and respiratory syndrome virus vaccine candidates.

Porcine reproductive and respiratory syndrome virus (PRRSV) is an emerging pathogen causing significant economic losses in the swine industry worldwide. Two novel gene-deleted viruses were constructed and evaluated as vaccine candidates. Using the full-length infectious cDNA clone of North American PRRS isolate P129, the ORF2 and ORF4 genes (which encoded minor structural glycoproteins GP2a/2b and GP4, respectively) were individually deleted from the viral genome.

The development and characterization of an in vitro model of psoriasis.

In this study, the phenotype of psoriatic keratinocytes and fibroblasts in reconstructed skin models was compared to those constructed from normal cells. Characterization of this model by immunohistochemistry showed that classical markers of keratinocyte differentiation exhibited similar patterns of distribution in the psoriatic models to those derived from normal cells and generally reflected in vivo observations. Some crucial differences, however, were observed between normal and psoriatic models when pro-inflammatory gene expression and keratinocyte proliferation were investigated.