Search results for "GENI"

showing 10 items of 6843 documents

Apert Syndrome With FGFR2 758 C > G Mutation: A Chinese Case Report

2018

Background: Apert syndrome is considered as one of the most common craniosynostosis syndromes with a prevalence of 1 in 65,000 individuals, and has a close relationship with point mutations in FGFR2 gene.Case report: Here, we described a Apert syndrome case, who was referred to genetic consultation in our hospital with the symptom of craniosynostosis and syndactyly of the hands and feet. Craniosynostosis, midfacial retrusion, steep wide forehead, larger head circumference, marked depression of the nasal bridge, short and wide nose and proptosis could be found obviously, apart from these, ears were mildly low compared with normal children and there was no cleft lip and palate. Mutation was i…

0301 basic medicinemusculoskeletal diseasesPediatricsmedicine.medical_specialtycongenital hereditary and neonatal diseases and abnormalitiesNasal bridgelcsh:QH426-470Case ReportApert syndromeCraniosynostosis03 medical and health sciencesExonsymbols.namesake0302 clinical medicineGeneticsmedicineSyndactylyGenetics (clinical)NoseSanger sequencingbusiness.industryPoint mutationmedicine.diseaseexons sequencingcraniosynostosislcsh:Genetics030104 developmental biologymedicine.anatomical_structureFGFR2genetic mutationsymbolsMolecular Medicinebusiness030217 neurology & neurosurgeryApert syndromeFrontiers in Genetics
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Therapeutic Potential of AntagomiR-23b for Treating Myotonic Dystrophy

2020

Myotonic dystrophy type 1 (DM1) is a chronically debilitating, rare genetic disease that originates from an expansion of a noncoding CTG repeat in the dystrophia myotonica protein kinase (DMPK) gene. The expansion becomes pathogenic when DMPK transcripts contain 50 or more repetitions due to the sequestration of the muscleblind-like (MBNL) family of proteins. Depletion of MBNLs causes alterations in splicing patterns in transcripts that contribute to clinical symptoms such as myotonia and muscle weakness and wasting. We previously found that microRNA (miR)-23b directly regulates MBNL1 in DM1 myoblasts and mice and that antisense technology (“antagomiRs”) blocking this microRNA (miRNA) boost…

0301 basic medicinemusculoskeletal diseasescongenital hereditary and neonatal diseases and abnormalitiesMyotonic dystrophyArticleantagomiR03 medical and health scienceschemistry.chemical_compound0302 clinical medicineDrug DiscoverymicroRNAMedicineMBNL1AntagomirProtein kinase AmiRNAmyotonic dystrophybusiness.industrylcsh:RM1-950Muscle weaknessmedicine.diseaseMyotoniaMbnl1030104 developmental biologylcsh:Therapeutics. Pharmacologychemistry030220 oncology & carcinogenesisRNA splicingCancer researchHSALR miceMolecular Medicinemedicine.symptomDM1antisense oligonucleotidesbusinessMolecular Therapy: Nucleic Acids
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A Standardized Wedelia chinensis Extract Overcomes the Feedback Activation of HER2/3 Signaling upon Androgen-Ablation in Prostate Cancer

2017

Crosstalk between the androgen receptor (AR) and other signaling pathways in prostate cancer (PCa) severely affects the therapeutic outcome of hormonal therapy. Although anti-androgen therapy prolongs overall survival in PCa patients, resistance rapidly develops and is often associated with increased AR expression and upregulation of the HER2/3-AKT signaling pathway. However, single agent therapy targeting AR, HER2/3 or AKT usually fails due to the reciprocal feedback loop. Previously, we reported that wedelolactone, apigenin, and luteolin are the active compounds in Wedelia chinensis herbal extract, and act synergistically to inhibit the AR activity in PCa. Here, we further demonstrated th…

0301 basic medicinenatural productsmedicine.drug_classPharmacologyurologic and male genital diseasesAntiandrogen03 medical and health scienceschemistry.chemical_compoundProstate cancer0302 clinical medicineHER3HER2LNCaPMedicineEnzalutamidePharmacology (medical)Pharmacologybusiness.industrylcsh:RM1-950apoptosisprostate cancerWedelolactonemedicine.diseaseAndrogenanimal modelsAndrogen receptorlcsh:Therapeutics. Pharmacology030104 developmental biologychemistry030220 oncology & carcinogenesisHormonal therapybusinessFrontiers in Pharmacology
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Transient hypothyroidism during lactation alters the development of the corpus callosum in rats. An in vivo magnetic resonance image and electron mic…

2020

Magnetic resonance imaging (MRI) data of children with late diagnosed congenital hypothyroidism and cognitive alterations such as abnormal verbal memory processing suggest altered telencephalic commissural connections. The corpus callosum (CC) is the major inter-hemispheric commissure that contra-laterally connects neocortical areas. However, in late diagnosed neonates with congenital hypothyroidism, the possible effect of early transient and chronic postnatal hypothyroidism still remains unknown. We have studied the development of the anterior, middle and posterior CC, using in vivo MRI and electron microscopy in hypothyroid and control male rats. Four groups of methimazole (MMI) treated r…

0301 basic medicineneocortical developmentmedicine.medical_specialtyNeuroscience (miscellaneous)autismattention deficit/hyperactivity disorderCorpus callosumNerve conduction velocitylcsh:RC321-571lcsh:QM1-695law.invention03 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicineIn vivolawInternal medicineLactationmedicinelcsh:Neurosciences. Biological psychiatry. NeuropsychiatryOriginal Researchthyroid hormonesiodine dietmedicine.diagnostic_testbusiness.industrycongenital hypothyroidismpsychiatric diseasesMagnetic resonance imaginglcsh:Human anatomyCommissuremedicine.diseaseCongenital hypothyroidismNeuroanatomy030104 developmental biologyEndocrinologymedicine.anatomical_structureAnatomyElectron microscopebusiness030217 neurology & neurosurgery
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Use of Early Life-Stages of Zebrafish to Assess Toxicity of Sediments Contaminated by Organotin Compounds

2016

ABSTRACTThis study examined the response of early life-stages (ELS) of zebrafish to organotin-contaminated sediment from Lake Huruslahti (HL) in Central Finland. A dilution series (0, 10, 33, and 100%) of the native (HL) and the sediment spiked with tributyltin (TBT) determined a dose-response of zebrafish ELS to organotin-contaminated sediment. Sediment elutriates were assessed by bacterial bioluminescence assay and microscopical pathologies of 1–3 days post-fertilization zebrafish (1–3dpfZF). Brain aromatase (cyp19a1b) and tissue vitellogenin (vtg1) were assayed from early-juvenile zebrafish (20dpfZF) exposed to intact sediment. In vivo modulation of cyp19a1b and vtg1 transcripts in 20dpf…

0301 basic medicineneural aromatase (cyp19a1b)Health Toxicology and Mutagenesista1172Soil Science010501 environmental sciences01 natural sciencessediment assay in vivotributyltin03 medical and health scienceschemistry.chemical_compoundVitellogeninIn vivoEnvironmental ChemistryBioluminescenceZebrafish0105 earth and related environmental sciencesbiologySedimentAquatic animalbiology.organism_classificationzebrafishPollutionMolecular biologyvitellogenin 1 (vtg1)030104 developmental biologychemistryEnvironmental chemistryToxicityTributyltinbiology.proteinSoil and Sediment Contamination
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Characterization of Differentially Expressed Circulating miRNAs in Metabolically Healthy versus Unhealthy Obesity

2021

Obese individuals without metabolic comorbidities are categorized as metabolically healthy obese (MHO). MicroRNAs (miRNAs) may be implicated in MHO. This cross-sectional study explores the link between circulating miRNAs and the main components of metabolic syndrome (MetS) in the context of obesity. We also examine oxidative stress biomarkers in MHO vs. metabolically unhealthy obesity (MUO). We analysed 3536 serum miRNAs in 20 middle-aged obese individuals: 10 MHO and 10 MUO. A total of 159 miRNAs were differentially expressed, of which, 72 miRNAs (45.2%) were higher and 87 miRNAs (54.7%) were lower in the MUO group. In addition, miRNAs related to insulin signalling and lipid metabolism pat…

0301 basic medicineobesitymedicine.medical_specialtyatherogenic dyslipidaemiaMedicine (miscellaneous)Context (language use)Biologymedicine.disease_causeArticlemetabolic syndromeGeneral Biochemistry Genetics and Molecular Biology03 medical and health sciences0302 clinical medicineInsulin resistanceDownregulation and upregulationinsulin resistanceInternal medicinemicroRNAmedicineoxidative stresslcsh:QH301-705.5Lipid metabolismmedicine.diseaseObesitymicroRNAs030104 developmental biologyEndocrinologylcsh:Biology (General)030220 oncology & carcinogenesisMetabolic syndromeOxidative stressBiomedicines
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The MID1 protein is a central player during development and in disease.

2015

Loss-of-function mutations in the MID1 gene cause a rare monogenic disorder, Opitz BBB/G syndrome (OS), which is characterized by malformations of the ventral midline. The MID1 gene encodes the MID1 protein, which assembles a large microtubule-associated protein complex. Intensive research over the past several years has shed light on the function of the MID1 protein as a ubiquitin ligase and regulator of mTOR signalling and translational activator. As a central player in the cell MID1 has been implicated in the pathogenesis of various other disorders in addition to OS including cancer and neurodegenerative diseases. Influencing the activity of the MID1 protein complex is a promising new st…

0301 basic medicinephysiopathology [Huntington Disease]CarcinogenesisUbiquitin-Protein LigasesRegulatorDiseaseBiologyBioinformaticsmedicine.disease_causephysiopathology [Alzheimer Disease]Congenital AbnormalitiesPathogenesis03 medical and health sciencesMiceAlzheimer Diseasephysiology [Nuclear Proteins]medicineAnimalsHumansgenetics [Microtubule Proteins]ddc:610GenePI3K/AKT/mTOR pathwayActivator (genetics)Nuclear Proteinsgenetics [Nuclear Proteins]genetics [Transcription Factors]physiology [Transcription Factors]Ubiquitin ligase030104 developmental biologyHuntington DiseaseMutationbiology.proteinMicrotubule Proteinsphysiology [Microtubule Proteins]CarcinogenesisMid1 protein humanTranscription FactorsFrontiers in bioscience (Landmark edition)
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The Impact of Biopreparations and Phytopathogenic Bacteria of the Pseudomonas Genus on L-Phenylalanine Ammonia-Lyase Activity in Soybean and Lupine P…

2020

It is known that plant protection against diseases is based mainly on the use of pesticides. These chemicals or their degradation products have a detrimental effect on the environment and human health. Due to this, the search for methods of plant protection that are safe for the environment is becoming increasingly popular. Induction of plant resistance to disease is one of the promising non-chemical ways of protection, in which plant enzymes play a key role. It was shown that in response to pathogen invasion, plants enhance protective properties by inducing the activity of a wide range of enzymes that slow the spread of infection, in particular: peroxidases, β-1,3-glucanases, chitinases, p…

0301 basic medicinephytopathogenic bacteria of the Pseudomonas genusdegree of damagebiologyChemistryfungiPseudomonasfood and beveragesGeneral MedicineL-phenylalanine ammonia-lyase activitybiology.organism_classificationplant resistance03 medical and health sciences030104 developmental biology0302 clinical medicineGenus030220 oncology & carcinogenesisL-phenylalanine ammonia-lyase activityBotanyBacteriaMikrobiolohichnyi Zhurnal
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Role of Ocular Angiogenic Factors in the Development of Neovascular Age-Related Macular Degeneration

2020

Abstract Age-related macular degeneration (AMD) is a progressive degenerative eye disease. Neovascular age-related macular degeneration (nAMD) is the advanced form of AMD characterised by abnormal growth of newly formed blood vessels in chorioidea which typically involves fluid accumulation in the retina or retinal haemorrhage, retinal epithelial detachments, hard exudate or subretinal scars. The process of angiogenesis is controlled by ocular angiogenic factors, which have enabled the development of different treatment options aimed at these factors. This review aims to compile the available information about the most commonly identified ocular angiogenic factors, uncovering their role in …

0301 basic medicinepigment epithelium-derived factormedicine.medical_specialtyMultidisciplinaryvascular endothelial growth factorgenetic structuresGeneral interestScienceangiogenic factorsQbiomarkersMacular degenerationmedicine.diseaseeye diseases03 medical and health sciences030104 developmental biology0302 clinical medicineAge relatedOphthalmology030221 ophthalmology & optometrymedicinesense organsProceedings of the Latvian Academy of Sciences. Section B. Natural, Exact, and Applied Sciences.
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In Vivo Cardiotoxicity Induced by Sodium Aescinate in Zebrafish Larvae

2016

Sodium aescinate (SA) is a widely-applied triterpene saponin product derived from horse chestnut seeds, possessing vasoactive and organ-protective activities with oral or injection administration in the clinic. To date, no toxicity or adverse events in SA have been reported, by using routine models (in vivo or in vitro), which are insufficient to predict all aspects of its pharmacological and toxicological actions. In this study, taking advantage of transparent zebrafish larvae (Danio rerio), we evaluated cardiovascular toxicity of SA at doses of 1/10 MNLC, 1/3 MNLC, MNLC and LC10 by yolk sac microinjection. The qualitative and quantitative cardiotoxicity in zebrafish was assessed at 48 h p…

0301 basic medicinesodium aescinateEmbryo NonmammalianHeart malformationDrug Evaluation PreclinicalPharmaceutical Science010501 environmental sciencesPharmacology01 natural sciencesAnalytical ChemistryHeart RateDrug DiscoveryToxicity Tests ChronicZebrafishYolk SacbiologyCommunicationHeartLC10medicine.anatomical_structureChemistry (miscellaneous)LarvaToxicityMolecular MedicineHeart Defects CongenitalMicroinjectionscardiotoxicityHemorrhagelarvaelcsh:QD241-44103 medical and health scienceslcsh:Organic chemistryIn vivoHeart ratemedicineMNLCAnimalsPhysical and Theoretical ChemistryYolk sacAdverse effect0105 earth and related environmental sciencesCardiotoxicityDose-Response Relationship DrugOrganic ChemistryThrombosisSaponinsbiology.organism_classificationzebrafishTriterpenes030104 developmental biologyMolecules
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