Primary Adult-Onset Hemophagocytic Lymphohistiocytosis with Neurologic Presentation.

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune deregulatory disorder that predominantly presents in children. Here we describe three patients with adult-onset primary HLH whose initial presentations were characterized by neurological features, and we review the literature of published cases. These cases ranged in age from 17 to 30 and presented with a variety of neurological symptoms.

Myotonic disorders: A review article.

The myotonic disorders are a heterogeneous group of genetically determined diseases that are unified by the presence of myotonia, which is defined as failure of muscle relaxation after activation. The presentation of these disorders can range from asymptomatic electrical myotonia, as seen in some forms of myotonia congenita (MC), to severe disability with muscle weakness, cardiac conduction defects, and other systemic features as in myotonic dystrophy type I (DM1). In this review, we describe the clinical features and pathophysiology of the different myotonic disorders, their laboratory and electrophysiologic findings and briefly review the currently available treatments.

Data Harmonization in Aging Research: Not so Fast.

Background/study Context: Harmonizing measures in order to conduct pooled data analyses has become a scientific priority in aging research. Retrospective harmonization where different studies lack common measures of comparable constructs presents a major challenge. This study compared different approaches to harmonization with a crosswalk sample who completed multiple versions of the measures to be harmonized.

Large-scale synthesis of transition-metal-doped TiO2 nanowires with controllable overpotential.

Practical implementation of one-dimensional semiconductors into devices capable of exploiting their novel properties is often hindered by low product yields, poor material quality, high production cost, or overall lack of synthetic control. Here, we show that a molten-salt flux scheme can be used to synthesize large quantities of high-quality, single-crystalline TiO2 nanowires with controllable dimensions. Furthermore, in situ dopant incorporation of various transition metals allows for the tuning of optical, electrical, and catalytic properties.

Photoelectrochemical properties of TiO2 nanowire arrays: a study of the dependence on length and atomic layer deposition coating.

We report that the length and surface properties of TiO(2) nanowires can have a dramatic effect on their photoelectrochemical properties. To study the length dependence, rutile TiO(2) nanowires (0.28-1.

Si/InGaN core/shell hierarchical nanowire arrays and their photoelectrochemical properties.

Three-dimensional hierarchical nanostructures were synthesized by the halide chemical vapor deposition of InGaN nanowires on Si wire arrays. Single phase InGaN nanowires grew vertically on the sidewalls of Si wires and acted as a high surface area photoanode for solar water splitting. Electrochemical measurements showed that the photocurrent density with hierarchical Si/InGaN nanowire arrays increased by 5 times compared to the photocurrent density with InGaN nanowire arrays grown on planar Si (1.

Durability of the effects of testosterone and growth hormone supplementation in older community-dwelling men: the HORMA Trial.

Objectives: To determine the durability of anabolic effects and adverse events (AEs) after stopping testosterone and growth hormone supplementation in older men.

Design: Secondary analysis of a double-masked, randomized controlled trial of testosterone gel (5 or 10 g/daily) plus rhGH (0, 3 or 5 μg/kg/day) with follow-up of outcomes 3 months later.

Participants: A total of 108 community-dwelling 65- to 90-year-old men.

JAM-C induces endothelial cell permeability through its association and regulation of {beta}3 integrins.

Objective: The molecular mechanisms regulating vascular permeability are only now being elucidated. The junctional adhesion molecule (JAM) JAM-C has been linked to the induction of vascular permeability. We sought to understand the mechanism whereby JAM-C may disrupt junctional integrity in endothelial cells (ECs).

Chronic increases in sphingosine kinase-1 activity induce a pro-inflammatory, pro-angiogenic phenotype in endothelial cells.

Sphingosine kinase-1 (SK1) promotes the formation of sphingosine-1-phosphate (S1P), which has potent pro-inflammatory and pro-angiogenic effects. We investigated the effects of raised SK1 levels on endothelial cell function and the possibility that this signaling pathway is activated in rheumatoid arthritis. Human umbilical vein endothelial cells with 3- to 5-fold SK1 (EC(SK)) overexpression were generated by adenoviral and retroviralmediated gene delivery.

Sphingosine kinase-1 enhances endothelial cell survival through a PECAM-1-dependent activation of PI-3K/Akt and regulation of Bcl-2 family members.

Sphingosine-1-phosphate (S1P), the bioactive product of sphingosine kinase (SK) activation, is a survival factor for endothelial cells. The mechanism of SK-mediated survival was investigated in endothelial cells with moderately raised intracellular SK activity. Overexpression of SK mediated survival primarily through the activation of the phosphatidyl inositol 3-kinase (PI-3K)/protein kinase B (Akt/PKB) pathway and an associated up-regulation of the antiapoptotic protein B cell lymphoma gene 2 (Bcl-2) and down-regulation of the proapoptotic protein bisindolylmaleimide (Bcl-2 interacting mediator of cell death; Bim).

Apolipoprotein E enhances hepatic lipase-mediated hydrolysis of reconstituted high-density lipoprotein phospholipid and triacylglycerol in an isoform-dependent manner.

This study compares the kinetics of hepatic lipase (HL)-mediated phospholipid and triacylglycerol hydrolysis in spherical, reconstituted high-density lipoproteins (rHDL) that contain either apolipoprotein E2 (apoE2), apoE3, apoE4, or apoA-I as the sole apolipoprotein. HL-mediated phospholipid hydrolysis was assessed by incubating various concentrations of rHDL that contained only cholesteryl esters (CE) in their core, (E2/CE)rHDL, (E3/CE)rHDL, (E4/CE)rHDL, and (A-I/CE)rHDL, with a constant amount of HL. The rate of phospholipid hydrolysis was determined as the formation of nonesterified fatty acid mass.

PPARgamma agonists ameliorate endothelial cell activation via inhibition of diacylglycerol-protein kinase C signaling pathway: role of diacylglycerol kinase.

Subject- Peroxisome proliferator-activated receptor (PPAR)-gamma agonists are emerging as potential protectors against inflammatory cardiovascular diseases including atherosclerosis and diabetic complications. However, their molecular mechanism of action within vasculature remains unclear. We report here that PPARgamma agonists, thiazolidinedione class drugs (TZDs), or 15-deoxy-Delta12,14-prostaglandin J2 (15d-PGJ2) were capable of activating diacylglycerol (DAG) kinase (DGK), resulting in attenuation of DAG levels and inhibition of protein kinase C (PKC) activation.