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https://www.readbyqxmd.com/read/28238808/hypophosphatasia-an-overview-for-2017
#1
Michael P Whyte
Hypophosphatasia (HPP) is the inborn-error-of-metabolism that features low serum alkaline phosphatase (ALP) activity (hypophosphatasemia) caused by loss-of-function mutation(s) of the gene that encodes the tissue-nonspecific isoenzyme of ALP (TNSALP). Autosomal recessive or autosomal dominant inheritance from among >300 TNSALP (ALPL) mutations largely explains HPP's remarkably broad-ranging severity. TNSALP is a cell-surface homodimeric phosphohydrolase richly expressed in the skeleton, liver, kidney, and developing teeth...
February 24, 2017: Bone
https://www.readbyqxmd.com/read/28221934/effect-of-process-temperature-on-virus-inactivation-during-high-hydrostatic-pressure-processing-of-contaminated-fruit-puree-and-juice
#2
Hao Pan, Matthew Buenconsejo, Karl F Reineke, Y Carol Shieh
High pressure processing (HPP) can inactivate pathogens and retain fruit qualities. Elevated HPP pressure or time increases virus inactivation, but the effect of temperature is not consistently observed for norovirus and hepatitis A virus. In the present study, the effectiveness of HPP holding temperatures (<40°C) and pressures were evaluated for inactivating surrogates (murine norovirus [MNV] and MS2 coliphage) in pomegranate and strawberry juices and strawberry puree using a 24-liter HPP system. The holding temperature was established by setting the HPP initial temperature via pretrials...
September 2016: Journal of Food Protection
https://www.readbyqxmd.com/read/28193435/effect-of-harvest-time-on-some-in-vitro-functional-properties-of-hop-polyphenols
#3
Takako Inui, Koharu Okumura, Hiroo Matsui, Takahiro Hosoya, Shigenori Kumazawa
Health benefits of hop polyphenols are well studied. In the present investigation, we examined the antioxidant activity, anti-nitric oxide (NO) production, and anti-adipocyte differentiation of polyphenols extracted from hops (HPP), harvested at different times for over 4years. Saaz hop variety from the Czech Republic was used in the in vitro assays. Twenty-three polyphenols were quantitatively analyzed using Orbitrap liquid chromatography-mass spectrometry (LC-MS), and their health promoting effects were assayed individually...
June 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28127875/molecular-and-clinical-analysis-of-alpl-in-a-cohort-of-patients-with-suspicion-of-hypophosphatasia
#4
Jair Tenorio, Ignacio Álvarez, Leyre Riancho-Zarrabeitia, Gabriel Á Martos-Moreno, Giorgia Mandrile, Monserrat de la Flor Crespo, Mikhail Sukchev, Mostafa Sherif, Iza Kramer, María T Darnaude-Ortiz, Pedro Arias, Gema Gordo, Irene Dapía, Julián Martinez-Villanueva, Rubén Gómez, José Manuel Iturzaeta, Ghada Otaify, Mayte García-Unzueta, Alessandro Rubinacci, José A Riancho, Mona Aglan, Samia Temtamy, Mohamed Abdel Hamid, Jesús Argente, Víctor L Ruiz-Pérez, Karen E Heath, Pablo Lapunzina
Hypophosphatasia (HPP) is a rare autosomal dominant or recessive metabolic disorder caused by mutations in the tissue nonspecific alkaline phosphatase gene (ALPL). To date, over 300 different mutations in ALPL have been identified. Disease severity is widely variable with severe forms usually manifesting during perinatal and/or infantile periods while mild forms are sometimes only diagnosed in adulthood or remain undiagnosed. Common clinical features of HPP are defects in bone and tooth mineralization along with the biochemical hallmark of decreased serum alkaline phosphatase activity...
January 27, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28094228/health-promotion-in-practice-district-nurses-experiences-of-working-with-health-promotion-and-lifestyle-interventions-among-patients-at-risk-of-developing-cardiovascular-disease
#5
Kristina Lundberg, Miek C Jong, Lisbeth Kristiansen, Mats Jong
OBJECTIVE: Health Promotion Practice (HPP) has the objective to promote a healthier lifestyle and reduce the risk of disease. The aim of this study was to examine district nurses׳ experiences of working with health preventive actions among patients with risk factors for cardiovascular disease (CVD), and to identify facilitators and obstacles in HPP. DESIGN/SETTING: The study was carried out with a qualitative approach where individual semistructured interviews were performed with a total of 12 district nurses in primary care...
December 16, 2016: Explore: the Journal of Science and Healing
https://www.readbyqxmd.com/read/28088025/residues-of-2-hydroxy-3-phenylpyrazine-a-degradation-product-of-some-%C3%AE-lactam-antibiotics-in-environmental-water-in-vietnam
#6
Nguyen Van Sy, Kazuo Harada, Megumi Asayama, Minae Warisaya, Le Hong Dung, Yoshinori Sumimura, Khong Thi Diep, Le Viet Ha, Nguyen Nam Thang, Tran Thi Tuyet Hoa, Tran Minh Phu, Pham Ngoc Khai, Nguyen Thanh Phuong, Le Danh Tuyen, Yoshimasa Yamamoto, Kazumasa Hirata
Antibiotic-resistant bacteria have become a serious problem worldwide, caused in part by the excessive use and discharge of antibiotics into the environment. Ampicillin (ABPC) is a widely used antibiotic. However, this chemical rapidly decomposes in water containing divalent cations like Ca(2+) and Mg(2+), thus, detection of ABPC in environmental water is difficult. This study was carried out to evaluate the presence of 2-hydroxy-3-phenylpyrazine (HPP), one of the degradation products of ABPC and β-lactam antibiotics with an ABPC substructure, in environmental water...
April 2017: Chemosphere
https://www.readbyqxmd.com/read/28072448/identification-of-altered-brain-metabolites-associated-with-tnap-activity-in-a-mouse-model-of-hypophosphatasia-using-untargeted-nmr-based-metabolomics-analysis
#7
Thomas Cruz, Marie Gleizes, Stéphane Balayssac, Etienne Mornet, Grégory Marsal, José Luis Millán, Myriam Malet-Martino, Lionel G Nowak, Véronique Gilard, Caroline Fonta
Tissue Nonspecific Alkaline Phosphatase (TNAP) is a key player of bone mineralization and TNAP gene (ALPL) mutations in human are responsible for hypophosphatasia (HPP), a rare heritable disease affecting the mineralization of bones and teeth. Moreover, TNAP is also expressed by brain cells and the severe forms of HPP are associated with neurological disorders, including epilepsy and brain morphological anomalies. However TNAP's role in the nervous system remains poorly understood. In order to investigate its neuronal functions, we aimed to identify without any a priori the metabolites regulated by TNAP in the nervous tissue...
January 10, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28071938/tailoring-structure-and-technological-properties-of-plant-proteins-using-high-hydrostatic-pressure
#8
Rui P Queirós, Jorge A Saraiva, José A Lopes da Silva
The demand for proteins is rising and alternatives to meat proteins are necessary since animal husbandry is expensive and intensive to the environment. Plant proteins appear as an alternative; however, their techno-functional properties need improvement. High-pressure processing (HPP) is a non-thermal technology that has several applications including the modification of proteins. The application of pressure allows modifying proteins' structure hence allowing to change several of their properties, such as hydration, hydrophobicity, and hydrophilicity...
January 10, 2017: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/28040169/high-pressure-inactivation-of-brettanomyces-bruxellensis-in-red-wine
#9
Sanelle van Wyk, Filipa V M Silva
Brettanomyces bruxellensis ("Brett") is a major spoilage concern for the wine industry worldwide, leading to undesirable sensory properties. Sulphur dioxide, is currently the preferred method for wine preservation. However, due to its negative effects on consumers, the use of new alternative non-thermal technologies are increasingly being investigated. The aim of this study was to determine and model the effect of high pressure processing (HPP) conditions and yeast strain on the inactivation of "Brett" in Cabernet Sauvignon wine...
May 2017: Food Microbiology
https://www.readbyqxmd.com/read/28000043/molecular-defect-of-tissue-nonspecific-alkaline-phosphatase-bearing-a-substitution-at-position-426-associated-with-hypophosphatasia
#10
Hiba A Al-Shawafi, Keiichi Komaru, Kimimitsu Oda
Mutations in the ALPL gene encoding tissue-nonspecific alkaline phosphatase (TNSALP) cause hypophosphatasia (HPP), a genetic disorder characterized by deficiency of serum ALP and hypomineralization of bone and teeth. Three missense mutations for glycine 426 (by standard nomenclature) of TNSALP have been reported: cysteine (p.G426C), serine (p.G426S), and aspartate (p.G426D). We expressed TNSALP mutants carrying each missense mutation in mammalian cells. All three TNSALP mutants appeared on the cell surface like the wild-type (WT) TNSALP, although the cells expressing each TNSALP mutant exhibited markedly reduced ALP activity...
March 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/27998428/-mutation-analysis-for-two-hypophosphatasia-families-with-targeted-next-generation-sequencing
#11
Y Bai, N Liu, J Yang, Y Guo, X D Kong
Objective: To detect the mutations in alkaline phosphatase (ALPL) gene of two Chinese families with perinatal hypophosphatasia (HPP), in order to explore the mechanism of this condition. Methods: Next-generation sequencing (NGS) of osteology system panel was carried out for exome sequencing in the mothers of 2 HPP fetuses, who visited Prenatal Diagnosis Center of the First Affiliated Hospital of Zhengzhou University. Further polymerase chain reaction (PCR) and Sanger sequencing validation was performed in the parents, affected fetuses and 200 unrelated healthy individuals to verify the mutation sites...
December 13, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27979173/effects-of-high-hydrostatic-pressure-ultraviolet-light-c-and-far-infrared-treatments-on-the-digestibility-antioxidant-and-antihypertensive-activity-of-%C3%AE-casein
#12
Guanlan Hu, Yuanrong Zheng, Zhenmin Liu, Yang Xiao, Yun Deng, Yanyun Zhao
Alpha-casein is the most important bioactive protein in processing technologies. This study investigated the digestibility, antioxidant and antihypertensive activities of α-casein when treated by high hydrostatic pressure (HPP), ultraviolet light-C (UV-C), and far-infrared radiation (FIR). The in vitro digestibility was modified after treatments, especially after 5min/200MPa HHP treatment. Ultra-performance liquid chromatography-tandem mass spectrometry analysis showed that one 5min/200MPa HHP treatment resulted in the highest yield of peptides...
April 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/27977135/characterization-of-two-late-stage-enzymes-involved-in-fosfomycin-biosynthesis-in-pseudomonads
#13
Philip Olivares, Emily C Ulrich, Jonathan R Chekan, Wilfred A van der Donk, Satish K Nair
The broad-spectrum phosphonate antibiotic fosfomycin is currently in use for clinical treatment of infections caused by both Gram-positive and Gram-negative uropathogens. The antibiotic is biosynthesized by various streptomycetes, as well as by pseudomonads. Notably, the biosynthetic strategies used by the two genera share only two steps: the first step in which primary metabolite phosphoenolpyruvate (PEP) is converted to phosphonopyruvate (PnPy) and the terminal step in which 2-hydroxypropylphosphonate (2-HPP) is converted to fosfomycin...
February 17, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/27958538/perfect-vortex-in-three-dimensional-multifocal-array
#14
Duo Deng, Yan Li, Yanhua Han, Xiaoya Su, Jingfu Ye, Jianmin Gao, Qiaoqun Sun, Shiliang Qu
We proposed an approach for creating three-dimensional (3D) multifocal perfect vortices arrays by using a high numerical aperture objective. The position, orbital angular momentum states, number and diameter of the perfect vortices can be freely modulated by a special designed hybrid phase plate (HPP). HPP could be calculated by 3D phase shifting expression which is derived from Fourier transform theory of the Debye diffraction integral. Furthermore, we developed a novel pixel checkerboard method for adding phase information into the HPP...
December 12, 2016: Optics Express
https://www.readbyqxmd.com/read/27938859/cyanogenic-pseudomonas-spp-strains-are-concentrated-in-the-rhizosphere-of-alpine-pioneer-plants
#15
Tomaž Rijavec, Aleš Lapanje
HCN producing bacteria have previously been isolated from alpine mineral soil and their ecophysiology was presumed to be associated with mineral weathering. Nevertheless, the high ecological patchiness of the alpine environment calls for an extensive and detailed analysis of the spatial distribution of HCN producing bacterial populations and their associated weathering traits. Our results of such an analysis showed that primarily the rhizosphere of pioneer plants was rich in HPPs, harbouring the most potent HCN producers...
January 2017: Microbiological Research
https://www.readbyqxmd.com/read/27919850/detection-and-differentiation-of-early-hepatocellular-carcinoma-from-cirrhosis-using-ct-perfusion-in-a-rat-liver-model
#16
Jin-Ping Li, Guang-Long Feng, Da-Qing Li, Hai-Bo Wang, De-Li Zhao, Yong Wan, Hui-Jie Jiang
BACKGROUND: Functional imaging such as CT perfusion can detect morphological and hemodynamic changes in hepatocellular carcinoma (HCC). Pre-carcinoma and early HCC nodules are difficult to differentiate by observing only their hemodynamics changes. The present study aimed to investigate hemodynamic parameters and evaluate their differential diagnostic cut-off between pre-carcinoma and early HCC nodules using CT perfusion and receiver operating characteristic (ROC) curves. METHODS: Male Wistar rats were randomly divided into control (n=20) and experimental (n=70) groups...
December 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/27893237/emergence-of-japanese-infants-prosodic-preferences-in-infant-directed-vocabulary
#17
Akiko Hayashi, Reiko Mazuka
The article examines the role of infant-directed vocabulary (IDV) in infants language acquisition, specifically addressing the question of whether IDV forms that are not prominent in adult language may nonetheless be useful to the process of acquisition. Japanese IDV offers a good test case, as IDV characteristically takes a bisyllabic H(eavy)-L(ight) form that is rare in adult speech. In 5 experiments using the Headturn Preference Procedure (HPP), 8- to 10-month-old Japanese infants, but not 4- to 6-month-olds, were found to show a preference for bisyllabic H-L words over other types of words...
January 2017: Developmental Psychology
https://www.readbyqxmd.com/read/27885688/effect-of-high-pressure-processing-and-thermal-pasteurization-on-overall-quality-parameters-of-white-grape-juice
#18
Yin-Hsuan Chang, Sz-Jie Wu, Bang-Yuan Chen, Hsiao-Wen Huang, Chung-Yi Wang
BACKGROUND: The aim of this study was to investigate the microbial levels, physicochemical and antioxidant properties, and polyphenoloxidase (PPO) and peroxidase (POD) activity, as well as to conduct a sensory analysis of white grape juice treated with high pressure processing (HPP) and thermal pasteurization (TP) over a period of 20 days of refrigerated storage. RESULTS: Results showed that HPP treatment of 600 MPa and TP significantly reduced aerobic bacteria, coliforms, and yeast counts...
November 24, 2016: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/27885680/effect-of-high-hydrostatic-pressure-on-the-enzymatic-hydrolysis-of-bovine-serum-albumin
#19
Serena De Maria, Giovanna Ferrari, Paola Maresca
BACKGROUND: The extent of the enzymatic proteolysis mainly depends on the accessibility of the peptide bonds, which stabilize the protein structure. High Hydrostatic Pressure (HHP) process is able to induce, at certain operating conditions, protein displacement, thus suggesting that this technology can be used to modify protein resistance to the enzymatic attack. This work aims at investigating the mechanism of the enzymatic hydrolysis assisted by HHP performed at different processing conditions (pressure level, treatment time)...
November 24, 2016: Journal of the Science of Food and Agriculture
https://www.readbyqxmd.com/read/27847653/nondestructive-microcomputed-tomography-evaluation-of-mineral-density-in-exfoliated-teeth-with-hypophosphatasia
#20
Sachiko Hayashi-Sakai, Takafumi Hayashi, Makoto Sakamoto, Jun Sakai, Junko Shimomura-Kuroki, Hideyoshi Nishiyama, Kouji Katsura, Makiko Ike, Yutaka Nikkuni, Miwa Nakayama, Marie Soga, Taichi Kobayashi
Most cases of hypophosphatasia (HPP) exhibit early loss of primary teeth. Results of microcomputed tomography (micro-CT) analysis of teeth with HPP have rarely been reported. The purpose of the present study was to describe the mineral density distribution and mapping of exfoliated teeth from an HPP patient using micro-CT. Four exfoliated teeth were obtained from a patient with HPP. Enamel and dentin mineral densities of exfoliated teeth were measured on micro-CT. The mean values of enamel and dentin mineral densities in mandibular primary central incisors with HPP were 1...
2016: Case Reports in Dentistry
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