Prevalence of Anderson-Fabry disease in male patients with late onset hypertrophic cardiomyopathy

Background-Although studies have suggested that "late-onset" hypertrophic cardiomyopathy (HCM) may be caused by sarcomeric protein gene mutations, the cause of HCM in the majority of patients is unknown. This study determined the prevalence of a potentially treatable cause of hypertrophy, Anderson-Fabry disease, in a HCM referral population.Methods and Results-Plasma alpha-galactosidase A (alpha-Gal) was measured in 79 men with HCM who were diagnosed at greater than or equal to40 years of age (52.9 +/- 7.7 years; range, 40-71 years) and in 74 men who were diagnosed at <40 years (25.9 +/- 9.2 years; range, 8-39 years). Five patients (6.3%) with late-onset disease and 1 patient (1.4%) diagnosed at <40 years had low alpha-Gal activity. Of these 6 patients, 3 had angina, 4 were in New York Heart Association class 2, 5 had palpitations, and 2 had a history of syncope. Hypertrophy was concentric in 5 patients and asymmetric in 1 patient. One patient had left ventricular outflow tract obstruction. All patients with low alpha-Gal activity had alpha-Gal gene mutations.Conclusion-Anderson-Fabry disease should be considered in all cases of unexplained hypertrophy. Its recognition is important given the advent of specific replacement enzyme therapy

Association between tobacco and alcohol use among young adult bar patrons: a cross-sectional study in three cities

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Antiretroviral treatment can affect the release of NO and EDCF, but EDH in rat arteries

This journal suppl. entitled: EDHF 2012 - 10th Anniversary MeetingDespite improving clinical outcomes, highly active antiretroviral therapy (HAART) is an independent potential risk factor for cardiovascular diseases. Currently the recommended HAART regimen commonly comprises a protease inhibitor (PI) with ritonavir (RTV)-boosting or a nonnucleoside reverse transcriptase inhibitor (NNRTI), and two nucleoside reverse transcriptase inhibitors. The present study examined whether or not boosted lopin...postprin

The presence and activity of SP-D in porcine coronary endothelial cells depend on Akt/PI3K, Erk and nitric oxide and decrease after multiple passaging

Surfactant protein D (SP-D) mediates clearance of microorganisms and modulates inflammation in response to cytotoxic stimulation. It is present in various epithelia, but also in vascular smooth muscle and endothelial cells. Experiments were designed to determine whether or not SP-D is present in porcine coronary arterial endothelial cells and if so, to investigate the molecular mechanisms underlying this presence. The expression of SP-D, NO synthase, Akt 1/2 and Erk 1/2 proteins was determined in cultures at passages 1 (#1) and 4 (#4). SP-D in primary cells existed in three isoforms (37-38 kDa and 50 kDa). The 37-38 kDa SP-D forms were the dominant isoforms in the porcine endothelium and were prominent at #1 but partially lost at #4. Tumor necrosis factor-α (TNF-α) significantly augmented the level of SP-D expression at #1 but not at #4. The basal level of 37-38 kDa SP-D isoforms at #1 was reduced by L-NAME, wortmannin and PD 98059. The low basal expression at #4 could be increased by DETA NONOate (donor of NO) or insulin (activator of PI3K/Akt). The presence of nitric oxide synthase was reduced while that of Akt 1/2 and Erk 1/2 was increased at #4. In cells both at passages 1 and 4, TNF-α downregulated NO synthase and up-regulated p-Erk 1/2 protein. The present findings demonstrate the presence of SP-D in endothelial cells which is NO-, PI3K/Akt- and Erk-dependent. They suggest a protective role of SP-D in these cells. © 2008 Elsevier Ltd. All rights reserved.postprin

Short-scale turbulent fluctuations driven by the electron-temperature gradient in the national spherical torus experiment

Measurements with coherent scattering of electromagnetic waves in plasmas of the National Spherical Torus Experiment indicate the existence of turbulent fluctuations in the range of wave numbers k(perpendicular to)rho(e)=0.1-0.4, corresponding to a turbulence scale length nearly equal to the collisionless skin depth. Experimental observations and agreement with numerical results from a linear gyrokinetic stability code support the conjecture that the observed turbulence is driven by the electron-temperature gradient.X1155sciescopu

A review of the association between congestive heart failure and cognitive impairment.

Heart failure is a growing epidemic with an estimated 5 million Americans suffering from this condition. Several clinical trials have demonstrated a high correlation between congestive heart failure (CHF) and cognitive impairment. The severity of cognitive impairment correlates positively with the degree of CHF. The underlying mechanism for cognitive impairment remains unclear but appears to be related to cerebral hypoperfusion and impaired cerebral reactivity with selective impairment of verbal memory and attention domains. Furthermore, cognitive dysfunction represents one aspect of frailty, a novel concept that encompasses a range of clinical conditions that results in functional impairment in patients with heart failure. In addition, frailty independently predicts mortality in CHF patients. Cognitive impairment is a common and predictable effect of CHF that contributes with social and behavioral problems to decreased compliance to prescribed therapy and increased hospital readmissions. A multidisciplinary approach is necessary to deal with the complexity of this clinical syndrome

Phosphorylation of nucleophosmin at threonine 234/237 is associated with HCC metastasis

Hepatocellular carcinoma (HCC) is frequently complicated by the occurrence of intrahepatic and extrahepatic metastases, leading to poor prognosis. To improve the prognosis for HCC patients, there is an urgent need to understand the molecular mechanisms of metastasis in HCC. Since protein Serine/Threonine phosphorylation emerges to be an important posttranslational modification critical in signaling process associated with cell proliferation, survival and metastasis, we employed a pair of primary tumor-derived and corresponding lung-metastatic counterparts (PLC/PRF/5-PT and PLC/PRF/5-LM) and aimed to identify these changes using CelluSpot Serine/Threonine kinase peptide array. Upon analysis, we found phosphorylated level of nucleophosmin (NPM) at Threonine 234/237 (p-NPM-Thr234/237) had remarkably high level in metastatic HCC cells (PLC-LM) than the corresponding primary HCC cell line (PLC-PT). Similar observation was observed in another match primary and their metastatic counterparts (MHCC-97L and MHCC-97H). By immunohistochemical staining, p-NPM-Thr234/237 was consistently found to be preferentially expressed in metastatic HCCs when compared with primary HCC in 28 HCC cases (p < 0.0001). By overexpressing Flag-tagged NPM and its phosphorylation site mutant (Thr234/237A) into low p-NPM-Thr234/237 expressing cells (Hep3B and Huh7) using a lentiviral based approach, we demonstrated that p-NPM-Thr234/237 is critical in invasion and migration of HCC cells, and this effect was mediated by cyclin-dependent kinase 1 (CDK1). Wild-type NPM was found to physically interact with a metastatic gene, ROCK2, and defective in Thr234/237 phosphorylation decreased its binding affinity, resulting in decrease in ROCK2 mediated signaling pathway. Identification of CDK1/p-NPM/ROCK2 signaling pathway provides a novel target for molecular therapy against HCC metastasis.published_or_final_versio

Unhealthy weight control behaviours in adolescent girls: a process model based on self-determination theory

This study used self-determination theory (Deci, E.L., & Ryan, R.M. (2000). The 'what' and 'why' of goal pursuits: Human needs and the self-determination of behavior. Psychological Inquiry, 11, 227-268.) to examine predictors of body image concerns and unhealthy weight control behaviours in a sample of 350 Greek adolescent girls. A process model was tested which proposed that perceptions of parental autonomy support and two life goals (health and image) would predict adolescents' degree of satisfaction of their basic psychological needs. In turn, psychological need satisfaction was hypothesised to negatively predict body image concerns (i.e. drive for thinness and body dissatisfaction) and, indirectly, unhealthy weight control behaviours. The predictions of the model were largely supported indicating that parental autonomy support and adaptive life goals can indirectly impact upon the extent to which female adolescents engage in unhealthy weight control behaviours via facilitating the latter's psychological need satisfaction

Consecutive treatment with phytase and arazyme influence protein hydrolysis of soybean meal

Soybean meal (SBM) is the main protein supplement used in animal feed worldwide. The degree of hydrolysis (DH) of SBM treated with two enzymes viz. phytase and arazyme was investigated for the first time in this study. The DH of SBM in the treatment with arazyme increased significantly as compared to the control without enzyme application. About 1.5-times and 10-fold higher DH were observed in phytase treatment when compared to the control treatments containing no enzyme. At the end of 24 h, enzymatic hydrolysis was done through consecutive treatment with 0.5% (w/v) phytase and 0.02% (w/v) arazyme, and the protein in the hydrolysate were mostly degraded free amino acids and peptides (&lt;6 KDa) when SDS-PAGE and fast protein liquid chromatography (FPLC) techniques used. Free amino acids contents of the soybean meal treated with phytase-arazyme increased by 2 to 14 fold as compared to products without enzyme. These results suggested that soybean meal proteins continuously treated with phytase and arazyme can be used as commercial feed additive for accelerated livestock growth.Key words: Soybean meal, phytase, arazyme, hydrolysis