47 research outputs found

    The Pah-R261Q mouse reveals oxidative stress associated with amyloid-like hepatic aggregation of mutant phenylalanine hydroxylase

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    Phenylketonuria (PKU) is caused by autosomal recessive variants in phenylalanine hydroxylase (PAH), leading to systemic accumulation of L-phenylalanine (L-Phe) that may reach neurotoxic levels. A homozygous Pah-R261Q mouse, with a highly prevalent misfolding variant in humans, reveals the expected hepatic PAH activity decrease, systemic L-Phe increase, L-tyrosine and L-tryptophan decrease, and tetrahydrobiopterin-responsive hyperphenylalaninemia. Pah-R261Q mice also present unexpected traits, including altered lipid metabolism, reduction of liver tetrahydrobiopterin content, and a metabolic profile indicative of oxidative stress. Pah-R261Q hepatic tissue exhibits large ubiquitin-positive, amyloid-like oligomeric aggregates of mutant PAH that colocalize with selective autophagy markers. Together, these findings reveal that PKU, customarily considered a loss-of-function disorder, can also have toxic gain-of-function contribution from protein misfolding and aggregation. The proteostasis defect and concomitant oxidative stress may explain the prevalence of comorbid conditions in adult PKU patients, placing this mouse model in an advantageous position for the discovery of mutation-specific biomarkers and therapies.publishedVersio

    Prediction well-being on basic components of hardiness

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    AbstractThe aim of this study is to review hardiness components contribution (commitment, control, challenge) in illuminating wellbeing. The study sample 50 managers and administrators of the Islamic Azad University (Khorasans’ branches) which were selected by using available sampling. This research instruments include the Personal View Survey (PVS) and well-being subscale of California Psychological Inventory. This research is from correlation type which Pearson's correlation coefficient and liner regression is used. Results show that hardiness predicts 38.1% and control component predicts 36.7% of the variables related to the well-being in a significant manner, in other words increase or decrease in manager's hardiness and control have significant varieties in relation to their well-being

    Relationship Between Brain/Behavioural Systems and Mental Health Among Students

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    AbstractThe purpose of this study was the examination relationship between the activity systems of brain/behavioural and mental health in students. This search was descriptive and from correlation kind. 100 students were selected by convenience sampling method. The participants were asked to complete the Gray-Wilson personality Questionnaire (GWPQ) and mental Health Questionnaire (DASS-21). Pearson's correlation coefficient and liner regression was used for data analyses.Results indicated there is a significant correlation between systems of brain/behavioural (BAS, BIS, FFS) and mental health. Results also indicated that systems of brain/behavioural (BAS, BIS, FFS) can predict mental health (Depression, anxiety, stress) in other word, increase or decrease the amount of the activity systems of brain/behavioural have significant varieties in relation to their mental health

    Curing Effectiveness of Bulk-fill Composites

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    Comparing the activity of brain/behavioral systems and happiness in male and female students

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    AbstractThis study aimed to compare the activity of brain/behavioral systems (behavioral activity system, behavioral inhibition system, fight-flight system) and happiness in male and female students. In the present study, 100 students (55 female and 45 male) were selected by convenience sampling method. The participants were asked to complete the Gray-Wilson personality Questionnaire (GWPQ) and oxford happiness inventory (0HI). Analysis of the data involved both descriptive and inferential statistics including means, standard deviations and Student's t-test. The results show there is a significant difference between two groups in level activity of behavioral activity system (BAS), behavioral inhibition system (BIS) and fight- flight system (FFS). Generally, happiness rate and activity level of BAS in males group is more than females group, but females group were more active in BIS and FFS. The results show that happiness rate is increased by more active BAS. Accordingly, the findings of this study conform with Gray model abut reinforcement sensitivity theory (RST)

    Beverage Influence on Direct Restorations

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    Phenylalanine Hydroxylase Misfolding and Pharmacological Chaperones

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    Composite Diametral Tensile Strength

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    A Face Recognition System Based on Principal Component Analysis-Wavelet and Support Vector Machines

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    Face recognition can represent a key requirement in various types of applications such as human-computer interface, monitoring systems, as well as personal identification. In this paper, design and implement of face recognition system are introduced. In this system, a combination of principal component analysis (PCA) and wavelet feature extraction algorithms with support vector machine (SVM) and K-nearest neighborhood classifier is used. PCA and wavelet transform methods are used to extract features from face image using and identify the image of the face using SVMs classifier as well as the neural network are used as a classifier to compare its results with the proposed system. For a more comprehensive comparison, two face image databases (Yale and ORL) are used to test the performance of the system. Finally, the experimental results show the efficiency and reliability of face recognition system, and the results demonstrate accuracy on two databases indicated that the results enhancement 5% using the SVM classifier with polynomial Kernel function compared to use feedforward neural network classifier.</jats:p
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