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https://www.readbyqxmd.com/read/28822915/immunomodulatory-effects-of-m2000-%C3%AE-d-mannuronic-acid-on-tnf-%C3%AE-il-17-and-foxp3-gene-expression-in-patients-with-inflammatory-bowel-disease
#1
Hussaini Alhassan Mohammed, Ali Akbar Saboor-Yaraghi, Homayoun Vahedi, Mir Saeed Yekaninejad, Ghodratollah Panahi, Gholamreza Hemmasi, Mostafa Lakzaei, Abbas Mirshafiey
INTRODUCTION: Inflammatory bowel diseases (IBD) are immune-mediated disorders that result from an aberrant immunological response to the gut luminal antigen in genetically susceptible patients. IBD is categorized into two serotype, Crohn's diseases (CD) and ulcerative colitis (UC), both subtype are important cause of gastrointestinal diseases. The increasing rate of hospitalization, with the high economic burden experienced by the IBD patients, calls for more concerted research efforts to design a potent and affordable treatment option for the treatment of IBD...
August 17, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28822816/genetically-modified-rabies-virus-vectored-ebola-virus-disease-vaccines-are-safe-and-induce-efficacious-immune-responses-in-mice-and-dogs
#2
Lei Shuai, Xijun Wang, Zhiyuan Wen, Jinying Ge, Jinliang Wang, Dandan Zhao, Zhigao Bu
Ebola viruses (EBOVs) are zoonotic pathogens that cause EBOV disease (EVD) with high case fatality in humans. Currently, EVD vaccines are still under development in several countries. Here, we generated two recombinant rabies viruses (RABVs), rERAG333E/ZGP and rERAG333E/SGP, expressing the Zaire EBOV glycoprotein (ZGP) or Sudan EBOV glycoprotein (SGP) gene based on a modified ERA vaccine strain (rERAG333E) vector platform. The recombinant RABVs retained growth properties similar to those of the vector virus in BSR cell culture and efficiently expressed ZGP or SGP...
August 16, 2017: Antiviral Research
https://www.readbyqxmd.com/read/28822792/imaging-assessment-of-fibrodysplasia-ossificans-progressiva-qualitative-quantitative-and-questionable
#3
Mona Al Mukaddam, Chamith S Rajapakse, Robert J Pignolo, Frederick S Kaplan, Stacy E Smith
Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare autosomal dominant genetic disorder of heterotopic ossification (HO) characterized by skeletal anomalies and episodic soft tissue swelling (flare-ups) that can transform into heterotopic bone. The progressive development of heterotopic bone and progressive arthropathy leads to significant limitation of mobility. This paper will review various imaging modalities used in evaluating episodic soft tissue swelling (flare-ups), heterotopic bone and skeletal anomalies...
August 16, 2017: Bone
https://www.readbyqxmd.com/read/28822770/regulation-of-autoimmune-myocarditis-by-host-responses-to-the-microbiome
#4
Jobert G Barin, Monica V Talor, Nicola L Diny, SuFey Ong, Julie A Schaub, Elizabeth Gebremariam, Djahida Bedja, Guobao Chen, Hee Sun Choi, Xuezhou Hou, Lei Wu, Ashley B Cardamone, Daniel A Peterson, Noel R Rose, Daniela Čiháková
The extensive, diverse communities that constitute the microbiome are increasingly appreciated as important regulators of human health and disease through inflammatory, immune, and metabolic pathways. We sought to elucidate pathways by which microbiota contribute to inflammatory, autoimmune cardiac disease. We employed an animal model of experimental autoimmune myocarditis (EAM), which results in inflammatory and autoimmune pathophysiology and subsequent maladaptive cardiac remodeling and heart failure. Antibiotic dysbiosis protected mice from EAM and fibrotic cardiac dysfunction...
August 16, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/28822768/functional-dissection-and-transport-mechanism-of-magnesium-in-plants
#5
REVIEW
Zhi Chang Chen, Wen Ting Peng, Jian Li, Hong Liao
Magnesium (Mg) is the second most abundant cation in plants, and, as such, is involved in numerous physiological and biochemical processes, including photosynthesis, enzyme activation, and synthesis of nucleic acids and proteins. Due to its relatively small ionic radius and large hydrated radius, Mg binds weakly to soil and root surfaces, and thereby is easily leached from soil. Mg deficiency not only affects crop productivity and quality, but also contributes to numerous chronic human diseases. Therefore, Mg nutrition in plants is an important issue in nutrition and food security...
August 16, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28822752/comprehensive-analysis-of-tissue-wide-gene-expression-and-phenotype-data-reveals-tissues-affected-in-rare-genetic-disorders
#6
Ariel Feiglin, Bryce K Allen, Isaac S Kohane, Sek Won Kong
Linking putatively pathogenic variants to the tissues they affect is necessary for determining the correct diagnostic workup and therapeutic regime in undiagnosed patients. Here, we explored how gene expression across healthy tissues can be used to infer this link. We integrated 6,665 tissue-wide transcriptomes with genetic disorder knowledge bases covering 3,397 diseases. Receiver-operating characteristics (ROC) analysis using expression levels in each tissue and across tissues indicated significant but modest associations between elevated expression and phenotype for most tissues (maximum area under ROC curve = 0...
August 14, 2017: Cell Systems
https://www.readbyqxmd.com/read/28822439/-multisystemic-smooth-muscle-dysfunction-syndrome-in-children-a-case-report-and-literature-review
#7
Y L Zhou, Y Y Zhang, B L Cheng, D Xu, L F Tang, Z M Chen
Objective: To analyze the clinical characteristics and diagnosis of multisystemic smooth muscle dysfunction syndrome(MSMDS). Method: Clinical data of a case diagnosed as MSMDS and hospitalized in our hospital in July 2016 was retrospectively analyzed. Literature search was performed at databases of PubMed, Wanfang, China National Knowledge Infrastructure and VIP with the key words "multisystemic smooth muscle dysfunction syndrome" "ACTA2" . The literature retrieval was confined from January 1980 to November 2016...
August 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28822354/3d-brain-organoids-derived-from-pluripotent-stem-cells-promising-experimental-models-for-brain-development-and-neurodegenerative-disorders
#8
REVIEW
Chun-Ting Lee, Raphael M Bendriem, Wells W Wu, Rong-Fong Shen
Three-dimensional (3D) brain organoids derived from human pluripotent stem cells (hPSCs), including embryonic stem cells (ESCs) and induced pluripotent stem cells (iPSCs), appear to recapitulate the brain's 3D cytoarchitectural arrangement and provide new opportunities to explore disease pathogenesis in the human brain. Human iPSC (hiPSC) reprogramming methods, combined with 3D brain organoid tools, may allow patient-derived organoids to serve as a preclinical platform to bridge the translational gap between animal models and human clinical trials...
August 20, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28822237/beyond-genomics-targeting-the-epigenome-in-diffuse-large-b-cell-lymphoma
#9
REVIEW
Andrea Kühnl, David Cunningham, Ian Chau
After decades of intense research on genetic alterations in cancer and successful implementation of genetically-based targeted therapies, the field of cancer epigenetics is only beginning to be fully recognized. The discovery of frequent mutations in genes modifying the epigenome in diffuse large B-cell lymphoma (DLBCL) has highlighted the outstanding role of epigenetic deregulation in this disease. Identification of epigenetically-driven DLBCL subgroups and development of novel epigenetic drugs have rapidly advanced...
August 9, 2017: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/28822080/soluble-epoxide-hydrolase-inhibition-attenuates-mptp-induced-neurotoxicity-in-the-nigrostriatal-dopaminergic-system-involvement-of-%C3%AE-synuclein-aggregation-and-er-stress
#10
Hui-Ju Huang, Yi-Ting Wang, Hui-Ching Lin, Yi-Hsuan Lee, Anya Maan-Yuh Lin
Soluble epoxide hydrolase (sEH) is widely expressed in the mammalian brain and possesses dual enzymatic activities, including C-terminal epoxide hydrolase (C-EH) which degrades epoxyeicosatrienoic acid (EET), a beneficial arachidonic acid metabolite. In the present study, the neuroprotective effect of sEH inhibition on 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced neurodegeneration of nigrostriatal dopaminergic system was investigated using genetic and pharmacological approaches. MPTP (15 mg/kg) was intraperitoneally injected in sEH knockout (KO) mice and C57BL/6J mice as wild-type (WT) mice...
August 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28822050/nuances-of-morphology-in-myelodysplastic-diseases-in-the-age-of-molecular-diagnostics
#11
REVIEW
Aaron C Shaver, Adam C Seegmiller
Morphologic dysplasia is an important factor in diagnosis of myelodysplastic syndrome (MDS). However, the role of dysplasia is changing as new molecular genetic and genomic technologies take a more prominent place in diagnosis. This review discusses the role of morphology in the diagnosis of MDS and its interactions with cytogenetic and molecular testing. Recent changes in diagnostic criteria have attempted to standardize approaches to morphologic diagnosis of MDS, recognizing significant inter-observer variability in assessment of dysplasia...
August 18, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28822009/urolithiasis-as-an-extraarticular-manifestation-of-ankylosing-spondylitis
#12
REVIEW
Cengiz Korkmaz, Döndü Üsküdar Cansu, John A Sayer
Ankylosing spondylitis (AS) affects sacroiliac and axial joints as well as extraarticular organs, such as the eye, lung, bowel, and heart. Although examples of renal involvement in AS, such as IgA nephropathy, amyloidosis, and glomerulonephritis, have been reported, it has not been emphasized that urolithiasis is frequently formed in the clinical course of AS. Growing evidence indicates that urolithiasis may be observed in AS patients and is more frequent than other extraarticular features. In this review, we will discuss frequency and predictors of AS-related urolithiasis and summarize the possible underlying genetic and biochemical mechanisms...
August 18, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28821934/whole-exome-sequencing-to-identify-the-cause-of-congenital-sensorineural-hearing-loss-in-carriers-of-a-heterozygous-gjb2-mutation
#13
Thomas Parzefall, Alexandra Frohne, Martin Koenighofer, Andreas Kirchnawy, Berthold Streubel, Christian Schoefer, Klemens Frei, Trevor Lucas
Bi-allelic variations in the gap junction protein beta-2 (GJB2) gene cause up to 50% of cases of newborn hearing loss. Heterozygous pathogenic GJB2 variations are also fivefold overrepresented in idiopathic patient groups compared to the normal-hearing population. Whether hearing loss in this group is due to unidentified additional variations within GJB2 or variations in other deafness genes is unknown in most cases. Whole-exome sequencing offers an effective approach in the search for causative variations in patients with Mendelian diseases...
August 18, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28821923/ankg-hemizygous-mice-present-cognitive-impairment-and-elevated-anxiety-depressive-like-traits-associated-with-decreased-expression-of-gaba-receptors-and-postsynaptic-density-protein
#14
Cui Liu, Ling Zhang, Jie Wu, Xiaolong Sui, Yanfeng Xu, Lan Huang, Yunlin Han, Hua Zhu, Yanhong Li, Xiuping Sun, Chuan Qin
Recent genome-wide association studies (GWAS) of patient populations and genetic linkage assessments have demonstrated that the ankyrin-G (AnkG) gene is involved in neuropsychiatric disorders, including bipolar disorder, schizophrenia, and Alzheimer's disease, but it remains unclear how the genetic variants of AnkG contribute to neuropsychiatric disorders. Here, we generated AnkG hemizygous mice using the gene trapping approach. Homozygous AnkG was embryonically lethal. Western blotting and real-time polymerase chain reaction (qPCR) assessments of wild type (WT) and AnkG (+/-) mutant mice demonstrated a 50% reduction of ANKG levels, at the gene and protein levels, in AnkG hemizygous mice...
August 18, 2017: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/28821885/the-expression-profiles-of-mirna-mrna-of-early-response-in-genetically-improved-farmed-tilapia-oreochromis-niloticus-liver-by-acute-heat-stress
#15
Jun Qiang, Wen J Bao, Fan Y Tao, Jie He, Xia H Li, Pao Xu, Lan Y Sun
Genetically improved farmed tilapia (GIFT, Oreochromis niloticus) are commercially important fish that are cultured in China. GIFT are highly susceptible to diseases when exposed to high temperatures in summer. Better understanding the GIFT regulatory response to heat stress will not only help in determining the relationship between heat stress signalling pathways and adaption mechanisms, but will also contribute to breeding new high-temperature tolerant strains of GIFT. In this study, we built control (28 °C) and heat-treated (37...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28821877/association-of-matrix-gla-protein-gene-rs1800801-rs1800802-rs4236-polymorphism-with-vascular-calcification-and-atherosclerotic-disease-a-meta-analysis
#16
Kaixiang Sheng, Ping Zhang, Weiqiang Lin, Jun Cheng, Jiawei Li, Jianghua Chen
Association between the MGP gene rs1800801, rs1800802, rs4236 polymorphisms and vascular calcification and atherosclerotic disease was inconsistent. To clarify precise association, we performed this meta-analysis. Medline, Embase and China Knowledge Resource Integrated Database were systematically searched through December 2016. A total of 23 case-control studies, consisting of 5280 cases and 5773 controls, were included. The overall results suggested that the -7A polymorphism was associated with an increased risk for vascular calcification and atherosclerotic disease in the recessive model (OR = 1...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28821638/fluorescent-probes-for-selective-protein-labeling-in-lysosomes-a-case-of-%C3%AE-galactosidase-a
#17
Cornelius Bohl, Adam Pomorski, Susanne Seemann, Anne-Marie Knospe, Chaonan Zheng, Artur Krężel, Arndt Rolfs, Jan Lukas
Fluorescence-based live-cell imaging (LCI) of lysosomal glycosidases is often hampered by unfavorable pH and redox conditions that reduce fluorescence output. Moreover, most lysosomal glycosidases are low-mass soluble proteins that do not allow for bulky fluorescent protein fusions. We selected α-galactosidase A (GALA) as a model lysosomal glycosidase involved in Anderson-Fabry disease (AFD) for the current LCI approach. Examination of the subcellular localization of AFD-causing mutants can reveal the mechanism underlying cellular trafficking deficits...
August 15, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28821618/region-specific-protein-misfolding-cyclic-amplification-reproduces-brain-tropism-of-prion-strains
#18
Nicolas Privat, Etienne Levavasseur, Serfildan Yildirim, Samia Hannaoui, Jean-Philippe Brandel, Jean-Louis Laplanche, Vincent Béringue, Danielle Seilhean, Stéphane Haïk
Human prion diseases such as Creutzfeldt-Jakob disease are transmissible brain proteinopathies, characterized by the accumulation of a misfolded isoform of the host cellular prion protein (PrP) in the brain. According to the prion model, prions are defined as proteinaceous infectious particles composed solely of this abnormal isoform of PrP (PrPSc). Even in the absence of genetic material, various prion strains can be propagated in experimental models. They can be distinguished by the pattern of disease they produce and especially by the localization of PrPSc deposits within the brain and the spongiform lesions they induce...
August 15, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28821568/lrrk2-promotes-the-activation-of-nlrc4-inflammasome-during-salmonella-typhimurium-infection
#19
Weiwei Liu, Xia'nan Liu, Yu Li, Junjie Zhao, Zhenshan Liu, Zhuqin Hu, Ying Wang, Yufeng Yao, Aaron W Miller, Bing Su, Mark R Cookson, Xiaoxia Li, Zizhen Kang
Although genetic polymorphisms in the LRRK2 gene are associated with a variety of diseases, the physiological function of LRRK2 remains poorly understood. In this study, we report a crucial role for LRRK2 in the activation of the NLRC4 inflammasome during host defense against Salmonella enteric serovar Typhimurium infection. LRRK2 deficiency reduced caspase-1 activation and IL-1β secretion in response to NLRC4 inflammasome activators in macrophages. Lrrk2(-/-) mice exhibited impaired clearance of pathogens after acute S...
August 18, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28821557/selected-alkylating-agents-can-overcome-drug-tolerance-of-g0-like-tumor-cells-and-eradicate-brca1-deficient-mammary-tumors-in-mice
#20
Marina Pajic, Sohvi Blatter, Charlotte Guyader, Maaike Gonggrijp, Ariena Kersbergen, Aslı Küçükosmanoğlu, Wendy Sol, Rinske Drost, Jos Jonkers, Piet Borst, Sven Rottenberg
We aimed to characterize and target drug-tolerant BRCA1-deficient tumor cells that cause residual disease and subsequent tumor relapse.<br /><br />Experimental Design: We studied responses to various mono- and bifunctional alkylating agents in a genetically engineered mouse model for BRCA1/p53-mutant breast cancer. Due to the large intragenic deletion of the Brca1 gene, no restoration of BRCA1 function is possible, and therefore no BRCA1-dependent acquired resistance occurs. To characterize the cell cycle stage from which Brca1(-/-);p53(-/-) mammary tumors arise after cisplatin treatment, we introduced the fluorescent ubiquitination-based cell cycle indicator (FUCCI) construct into the tumor cells...
August 18, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
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