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https://www.readbyqxmd.com/read/28327087/nt5c2-novel-splicing-variant-expands-the-phenotypic-spectrum-of-spastic-paraplegia-spg45-case-report-of-a-new-member-of-thin-corpus-callosum-spg-subgroup
#1
Mahmoud F Elsaid, Khalid Ibrahim, Nader Chalhoub, Ahmed Elsotouhy, Noora El Mudehki, Alice Abdel Aleem
BACKGROUND: Hereditary Spastic Paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative diseases. Thin Corpus Callosum (TCC) associated HSP is a distinguished subgroup of complex forms. Purines and pyrimidine, the basic DNA and RNA components, are regulating the cell metabolism, having roles in signal transduction, energy preservation and cellular repair. Genetic defects in nucleotide metabolism related genes have been only recently implicated in brain and neurodegenerative diseases' pathogenesis...
March 21, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28326005/bioactivity-of-food-peptides-biological-response-of-rats-to-bovine-milk-whey-peptides-following-acute-exercise
#2
Carolina Soares Moura, Pablo Christiano Barboza Lollo, Priscila Neder Morato, Eder Muller Risso, Jaime Amaya-Farfan
Background: Several physiologically beneficial effects of consuming a whey protein hydrolysate (WPH) have been attributed to the greater availability of bioactive peptides. Aims: The aim was to investigate the effect of four branched-chain amino acid- (BCAA-)containing dipeptides, present in WPH, on immune modulation, stimulation of HSP expression, muscle protein synthesis, glycogen content, satiety signals and the impact of these peptides on the plasma free amino acid profiles. Methods: The animals were divided in groups: control (rest, without gavage), vehicle (water), L-isoleucyl-L-leucine (lle-Leu), L-leucyl-L-isoleucine (Leu-lle), L-valyl-Lleucine (Val-Leu), L-leucyl-L-valine (Leu-Val) and WPH...
2017: Food & Nutrition Research
https://www.readbyqxmd.com/read/28321764/protective-manifestation-of-bacoside-a-and-bromelain-in-terms-of-cholinesterases-gamma-amino-butyric-acid-serotonin-level-and-stress-proteins-in-the-brain-of-dichlorvos-intoxicated-mice
#3
Bharti Chaudhary, Renu Bist
The objective of the study was to evaluate the neuroprotective effects of bacoside A and bromelain against dichlorvos-incited toxicity. Healthy 6-8-week old, male Swiss mice were administered subacute doses of dichlorvos (40 mg/kg bw), bacoside A (5 mg/kg bw) and bromelain (70 mg/kg bw). AChE, BChE, GABA, serotonin and total protein content and their expressions were used for determination of toxic action of dichlorvos. Protective effects of bacoside A and bromelain were evaluated on the same parameters...
March 20, 2017: Cell Stress & Chaperones
https://www.readbyqxmd.com/read/28320954/cerberus-nodal-lefty-pitx-signaling-cascade-controls-left-right-asymmetry-in-amphioxus
#4
Guang Li, Xian Liu, Chaofan Xing, Huayang Zhang, Sebastian M Shimeld, Yiquan Wang
Many bilaterally symmetrical animals develop genetically programmed left-right asymmetries. In vertebrates, this process is under the control of Nodal signaling, which is restricted to the left side by Nodal antagonists Cerberus and Lefty. Amphioxus, the earliest diverging chordate lineage, has profound left-right asymmetry as a larva. We show that Cerberus, Nodal, Lefty, and their target transcription factor Pitx are sequentially activated in amphioxus embryos. We then address their function by transcription activator-like effector nucleases (TALEN)-based knockout and heat-shock promoter (HSP)-driven overexpression...
March 20, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28319143/toxicity-effects-of-di-2-ethylhexyl-phthalate-to-eisenia-fetida-at-enzyme-cellular-and-genetic-levels
#5
Tingting Ma, Wei Zhou, Li'ke Chen, Longhua Wu, Peter Christie, Haibo Zhang, Yongming Luo
Di-(2-ethylhexyl) phthalate (DEHP) is a dominant phthalic acid ester (PAE) that has aroused public concern due to its resistance to degradation and its toxicity as an endocrine-disrupting compound. Effects of different concentrations of DEHP on Eisenia fetida in spiked natural soil have been studied in the body of the earthworm by means of soil cultivation tests 7, 14, 21 and 28 days after exposure. The results indicated that, in general, superoxide dismutase (SOD) activity, malondialdehyde (MDA) content, metallothionein (MT) content, the expression of heat shock protein 70 (HSP 70) and all the tested geno-toxicity parameters are promoted as time elapses and with increasing concentration of DEHP...
2017: PloS One
https://www.readbyqxmd.com/read/28318792/halogen-free-organophosphorus-flame-retardants-caused-oxidative-stress-and-multixenobiotic-resistance-in-asian-freshwater-clams-corbicula-fluminea
#6
Saihong Yan, Huimin Wu, Jianhui Qin, Jinmiao Zha, Zijian Wang
Halogen-free organophosphorus flame retardants are widespread in aquatic environments. Although it has been documented that they affect the behavior and reproduction of aquatic species, researches investigating cellular detoxification and the defense system in bivalves are scarce. In this study, adult Asian clams (C. fluminea) were exposed to tris (2-butoxyethyl) phosphate (TBEP) and tributyl phosphate (TBP) at 20, 200, and 2000 μg/L for 28 d. The results showed no noticeable difference in siphoning behavior...
March 16, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/28318003/small-molecule-hedgehog-pathway-antagonists
#7
Trieu N Trinh, Eileen A McLaughlin, Christopher P Gordon, Ilana R Bernstein, Victoria J Pye, Kate A Redgrove, Adam McCluskey
Leveraging our quinolone-1-(2H)-one based Hedgehog signalling pathway (HSP) inhibitors we have developed two new classes of HSP inhibitors based on: l-tryptophan and benzo[1,3]dioxol-5-ylmethyl-[2-(1H-indol-3-yl)-ethyl]-amine. Synthesis of focused compound libraries identified six l-tryptophan based inhibitors, and two stimulators, of Gli at 10 μM compound concentration. 2,4-Dichloro-13 and indole 16 suppressed mRNA expression of Ptch1 in Shh LIGHT2 cells, with 13 suppressing and 16 stimulating Gli2 mRNA expression...
March 20, 2017: Organic & Biomolecular Chemistry
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#8
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28302200/-association-between-ctla-4-gene-polymorphism-and-henoch-sch%C3%A3-nlein-purpura-in-children
#9
Hong-Hong Hou, Yan-Ping Huang, Li Liu, Gai-Tao He
OBJECTIVE: To investigate the association between CTLA-4 gene polymorphism and Henoch-Schönlein purpura (HSP) in children. METHODS: Sixty children who were diagnosed with HSP were enrolled as the case group, consisting of 33 males and 27 females. Thirty healthy children were enrolled as the control group. The patients were further divided into HSP nephritis (HSPN) and non-HSPN groups (n=30 each) according to the presence or absence of nephritis. Polymerase chain reaction-restriction fragment length polymorphism was used to analyze the genotype and allele frequencies at +49 and -1722 loci...
March 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28302199/-changes-in-25-hydroxyvitamin-d3-level-in-children-with-henoch-sch%C3%A3-nlein-purpura
#10
Yuan-Da Zhang, Qing-Wei Dong, Rong-Min Li, Chao-Yu Ji, Yong-Tao Chu, Lei Ma, Yu Zhang
OBJECTIVE: To examine the changes in 25-hydroxyvitamin D3 [25-(OH)D3] level in children with Henoch-Schönlein purpura (HSP) and its clinical significance. METHODS: A total of 92 HSP children were included in this study, and were divided into HSP nephritis (HSPN) group (31 cases) and HSP group (61 cases) based on the presence or absence of HSPN. Alternatively, the patients were divided into purpura alone group (22 cases), purpura with joint symptoms group (joint symptom group, 24 cases), purpura with gastrointestinal symptoms group (gastrointestinal symptom group, 20 cases), and purpura with joint and gastrointestinal symptoms (mixed group, 26 cases) based on their clinical symptoms...
March 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28300987/the-rio-de-janeiro-municipality-s-services-portfolio-and-health-actions-in-primary-care-in-brazil
#11
Bianca Alves Salazar, Mônica Rodrigues Campos, Vera Lucia Luiza
This study aimed to identify the provision of actions and procedures by family health teams (FHSt), based on Rio de Janeiro Municipality's (MRJ) Health Services Portfolio (HSP) and the main factors associated with this provision, in the different population strata. Data from the National Program for Improving Access and Quality of Primary Healthcare were used and implemented at the national level into 17,202 FHSts from June to September 2012. Outcome variables were "FHSt belonging to MRJ" and "FHSt providing all nine CS-MRJ procedures"...
March 2017: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/28296551/the-ameliorative-effects-of-vinpocetine-on-apoptosis-and-hsp-70-expression-in-testicular-torsion-in-rats
#12
M F Sönmez, Ş Ozdemir, M Guzel, E Kaymak
Vinpocetine is a potent antioxidant and free radical scavenger. We investigated the effects of vinpocetine on torsion/detorsion (T/D) induced testicular damage, HSP-70 expression and germ cell apoptosis in rats. Sixty Wistar albino adult male rats were divided into five groups of 12. The groups comprised a control group, a sham treated group, a T/D group, a vinpocetine treated group, and a T/D plus vinpocetine treated group. The left testis of each rat was subjected to unilateral torsion followed by detorsion after 2 h...
2017: Biotechnic & Histochemistry: Official Publication of the Biological Stain Commission
https://www.readbyqxmd.com/read/28295203/pla2g6-mutations-associated-with-a-continuous-clinical-spectrum-from-neuroaxonal-dystrophy-to-hereditary-spastic-paraplegia
#13
Burcak Ozes, Nazan Karagoz, Rebecca Schüle, Adriana Rebelo, María-Jesús Sobrido, Florian Harmuth, Matthis Synofzik, Samuel Ignacio Pascual Pascual, Melek Colak, Beyza Ciftci-Kavaklioglu, Batuhan Kara, Andrés Ordóñez-Ugalde, Beatriz Quintáns, Michael A Gonzalez, Aysun Soysal, Stephan Zuchner, Esra Battaloglu
PLA2G6-associated neurodegeneration (PLAN) and hereditary spastic paraplegia (HSP) are two groups of heterogeneous neurodegenerative diseases. In this study, we report PLA2G6 gene mutations in three families from Turkey, Morocco, and Romania. Two affected Turkish siblings presenting HSP adds the disease to PLAN phenotypes. They were homozygous for the PLA2G6 missense c.2239C>T, p.Arg747Trp variant and the ages of onset were 9 and 21. Parkinsonism, dystonia or cognitive decline were not the clinical elements in these patients contrary to the cases that has been previously reported with the same variant, however, iron accumulation was evident in their cranial MRI...
March 13, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28291803/induction-of-hsp70-in-tumor-cells-treated-with-inhibitors-of-the-hsp90-activity-a-predictive-marker-and-promising-target-for-radiosensitization
#14
Vladimir A Kudryavtsev, Anna V Khokhlova, Vera A Mosina, Elena I Selivanova, Alexander E Kabakov
We studied a role of the inducible heat shock protein 70 (Hsp70) in cellular response to radiosensitizing treatments with inhibitors of the heat shock protein 90 (Hsp90) chaperone activity. Cell lines derived from solid tumors of different origin were treated with the Hsp90 inhibitors (17AAG, geldanamycin, radicicol, NVP-AUY922) or/and γ-photon radiation. For comparison, human cells of the non-cancerous origin were subjected to the same treatments. We found that the Hsp90 inhibitors yielded considerable radiosensitization only when they cause early and pronounced Hsp70 induction; moreover, a magnitude of radiosensitization was positively correlated with the level of Hsp70 induction...
2017: PloS One
https://www.readbyqxmd.com/read/28288970/mycobacterium-tuberculosis-cpn60-2-groel2-blocks-macrophage-apoptosis-via-interaction-with-mitochondrial-mortalin
#15
Sunil Joseph, Alex Yuen, Vijender Singh, Zakaria Hmama
Earlier studies suggested that Mycobacterium tuberculosis (Mtb) proteins exported within the host macrophage play an essential role in tuberculosis pathogenesis. In fact, Mtb proteins interact with and deactivate key regulators of many macrophage functions such as phago-lysosome fusion and antigen presentation, resulting in the intracellular persistence of pathogenic mycobacteria. Cpn60.2 is an abundant Mtb chaperone protein, restricted to cell cytoplasm and surface, that was reported to be essential for bacterial growth...
March 13, 2017: Biology Open
https://www.readbyqxmd.com/read/28285429/hypothermia-decreased-the-expression-of-heat-shock-proteins-in-neonatal-rat-model-of-hypoxic-ischemic-encephalopathy
#16
Byong Sop Lee, Euiseok Jung, Yeonjoo Lee, Sung-Hoon Chung
Hypothermia (HT) is a well-established neuroprotective strategy against neonatal hypoxic ischemic encephalopathy (HIE). The overexpression of heat shock proteins (HSP) has been shown to provide neuroprotection in animal models of stroke. We aimed to investigate the effect of HT on HSP70 and HSP27 expression in a neonatal rat model of HIE. Seven-day-old rat pups were exposed to hypoxia for 90 min to establish the Rice-Vannucci model and were assigned to the following four groups: hypoxic injury (HI)-normothermia (NT, 36 °C), HI-HT (30 °C), sham-NT, and sham-HT...
March 11, 2017: Cell Stress & Chaperones
https://www.readbyqxmd.com/read/28283579/mitochondrial-chaperone-hsp-60-regulates-anti-bacterial-immunity-via-p38-map-kinase-signaling
#17
Dae-Eun Jeong, Dongyeop Lee, Sun-Young Hwang, Yujin Lee, Jee-Eun Lee, Mihwa Seo, Wooseon Hwang, Keunhee Seo, Ara B Hwang, Murat Artan, Heehwa G Son, Jay-Hyun Jo, Haeshim Baek, Young Min Oh, Youngjae Ryu, Hyung-Jun Kim, Chang Man Ha, Joo-Yeon Yoo, Seung-Jae V Lee
Mitochondria play key roles in cellular immunity. How mitochondria contribute to organismal immunity remains poorly understood. Here, we show that HSP-60/HSPD1, a major mitochondrial chaperone, boosts anti-bacterial immunity through the up-regulation of p38 MAP kinase signaling. We first identify 16 evolutionarily conserved mitochondrial components that affect the immunity of Caenorhabditis elegans against pathogenic Pseudomonas aeruginosa (PA14). Among them, the mitochondrial chaperone HSP-60 is necessary and sufficient to increase resistance to PA14...
March 10, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28282882/maternal%C3%A2-consumption%C3%A2-of%C3%A2-hesperidin%C3%A2-and%C3%A2-naringin%C3%A2-flavanones%C3%A2-exerts%C3%A2-transient%C3%A2-effects%C3%A2-to%C3%A2-tibia%C3%A2-bone%C3%A2-structure%C3%A2-in%C3%A2-female%C3%A2-cd-1%C3%A2-offspring
#18
Sandra M Sacco, Caitlin Saint, Paul J LeBlanc, Wendy E Ward
Hesperidin (HSP) and naringin (NAR), flavanones rich in citrus fruits, support skeletal integrity in adult and aging rodent models. This study determined whether maternal consumption of HSP and NAR favorably programs bone development, resulting in higher bone mineral density (BMD) and greater structure and biomechanical strength (i.e., peak load) in female offspring. Female CD-1 mice were fed a control diet or a HSP + NAR diet five weeks before pregnancy and throughout pregnancy and lactation. At weaning, female offspring were fed a control diet until six months of age...
March 8, 2017: Nutrients
https://www.readbyqxmd.com/read/28281222/gene-silencing-of-selenoprotein-k-induces-inflammatory-response-and-activates-heat-shock-proteins-expression-in-chicken-myoblasts
#19
Ruifeng Fan, Haidong Yao, Changyu Cao, Xia Zhao, Ahmed Khalid, Jinxin Zhao, Ziwei Zhang, Shiwen Xu
In the present study, specific small interfering RNA (siRNA) for selenoprotein K (Selk) gene was designed and transfected into chicken myoblasts. Then, the expressions of inflammatory factors (including induced nitric oxide synthase [iNOS], nuclear factor-kappa B [NF-κB], heme-oxygenase-1 [HO-1], cyclooxygenase-2 [COX-2], and prostaglandin E synthase [PTGEs]), inflammation-related cytokines (including interleukin [IL]-1β, IL-6, IL-7, IL-8, IL-17, and interferon [IFN]-γ), and heat shock proteins (HSPs) (including HSP27, HSP40, HSP60, HSP70, and HSP90) were examined at 24 and 72 h after transfection...
March 9, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28278364/methyl-cpg-level-at-distal-part-of-heat-shock-protein-promoter-hsp70-exhibits-epigenetic-memory-for-heat-stress-by-modulating-recruitment-of-pou2f1-associated-nucleosome-remodeling-deacetylase-nurd-complex
#20
Tatiana Kisliouk, Tomer Cramer, Noam Meiri
Depending on its stringency, exposure to heat in early life leads to either resilience or vulnerability to heat stress later in life. We hypothesized that epigenetic alterations in genes belonging to the cell proteostasis pathways are attributed to long-term responses to heat stress. Epigenetic regulation of the mRNA expression of the molecular chaperone heat-shock protein (HSP) 70 (HSPA2) was evaluated in the chick hypothalamus during the critical period of thermal-control establishment on day 3 posthatch and during heat challenge on day 10...
March 9, 2017: Journal of Neurochemistry
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