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Limb-girdle muscular dystrophy types 2E and F are characterized by skeletal muscle weakness and often cardiomyopathy and are due to mutations in the genes encoding beta- and delta-sarcoglycan. We previously demonstrated that loss of sarcoglycans in smooth muscle leads to constrictions of the microvasculature that contributes to the cardiac phenotype. It is unclear how vasculature abnormalities aff

Neurofibromatosis is associated with mid-aortic dysplasia, renal artery aneurysms and renal artery ostial stenosis, or occlusions in about 1% of cases. We describe a novel approach to recanalize an occluded renal artery in a 10-year-old girl with neurofibromatosis and difficulty in pharmacologically controlling her hypertension. Normally, when reconstruction is required, an open operative techniqu

Introduction: Radiofrequency ablation of atrial fibrillation (AF) is associated with energy delivery on the posterior left atrial (LA) wall with small but significant risk of life-threatening complications. Anatomy of LA walls has been described, but wall thickness in patients with AF has not been studied systematically. The aim of the present study was to describe LA posterior wall thickness in p

During puberty the mammary gland develops from a rudimentary tree to a branched epithelial network of ducts which can support alveolar development and subsequent milk production during pregnancy and lactation. This process involves growth, proliferation, migration, branching, invasion, apoptosis and above all, tight regulation which allows these processes to take place simultaneously during the co

The bactericidal/permeability-increasing protein (BPI), stored in human neutrophil granulocytes, is cytotoxic against Gram-negative bacteria. Several genes related to BPI cluster on human chromosome 20 and on mouse chromosome 2, but expression and characterization of a BPI ortholog in the mouse have not been reported. We asked whether BPI is structurally and functionally conserved between humans a

This paper focuses on propagation of transient electromagnetic waves in waveguides of general cross section with perfectly conducting walls. The solution of the transient wave propagation problem relies on a wave splitting technique, which has been frequently used in direct and inverse scattering problems during the last decade. The field in the waveguide is represented as a time convolution of a

A simple biosensor for the detection of hydrogen peroxide in organic solvents has been do,eloped and Coupled to a flow injection analysis (FIA) system. Catalase was entrapped in polyacrylamide gel anti placed oil the surface of platinum (working electrode) fixed in a Teflon holder with Ag-wire (auxiliary electrode), followed by addition of filter paper soaked in KCl. The entrapped catalase gel was

The microstructure of organogels formed upon the addition of tiny amounts of water to a solution of lecithin in fatty acid esters (viz. isopropylpalmitate and ethyloleate) was investigated by means of molecular self-diffusion measurements. In both systems lecithin and water form disconnected cylindrical reverse micelles. The ternary phase map for the lecithin/water/isopropylpalmitate has been inve

Ribonucleotide reduction is the only known biological means for de novo production of deoxyribonucleotides, the building blocks of DNA. These are produced from ribonucleotides, the building blocks of RNA, and the direction of this reaction has been taken to support the idea that, in evolution, RNA preceded DNA as genetic material. However, an understanding of the evolutionary relationships among t

We have investigated the effects of the novel imidazoline compound (+)-2-(2-(4,5-dihydro-1H-imidazol-2-yl)-thiopene-2-yl-ethyl)pyridine (NNC77-0074) on stimulus-secretion coupling in isolated pancreatic alpha- and beta-cells. NNC77-0074 stimulated glucose-dependent insulin secretion in intact mouse pancreatic islets. No effect was observed at less than or equal to 2.5 mM glucose and maximal stimul

[GRAPHIC] Two independent synthetic routes, starting from 1,3-cyclohexadione, toward the 4-hydroxy bicyclo[2.2.2]octane-2,6-dione derivative 3 are described.

A systematic comparison of the effect of architectural modifications to the network structure on the internal microstructure of N-isopropylacrylamide (NIPA) based hydrogels showed that the addition of a second component to the network significantly increased the proportion of macropores in the network. The second components considered were short poly(N-isopropylacrylamide)(PNIPAM) chains grafted t

1. Net primary production (NPP) by terrestrial ecosystems appears to be proportional to absorbed photosynthetically active radiation (APAR) on a seasonal and annual basis. This observation has been used in 'diagnostic' models that estimate NPP from remotely sensed vegetation indices. In 'prognostic' process-based models carbon fluxes are more commonly integrated with respect to leaf area index ass

During liquefaction of the ejaculate, the semen coagulum proteins semenogelin I (SEMG1) and semenogelin II (SEMG2) are degraded to low molecular mass fragments by kallikrein-related peptidase 3 (KLK3), also known as prostate-specific antigen. Semenogelin molecules initiate their own destruction by chelating Zn2+ that normally would completely inhibit the proteolytic activity of KLK3. In a similar