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showing 10 items of 830 documents

DLG5 variants do not influence susceptibility to inflammatory bowel disease in the Scottish population

2005

Introduction: Recent data have suggested that specific haplotypic variants of the DLG5 gene on chromosome 10q23 may be associated with susceptibility to inflammatory bowel disease (IBD) in Germany. Haplotype D, notably characterised by the presence of a G→A substitution at nucleotide 113, was associated with susceptibility to Crohn’s disease (CD) whereas an extended haplotype A conferred protection. Aims: Association of DLG5 haplotypic variants with disease susceptibility, genotype-phenotype relationships, and epistasis with CARD15 was investigated in the Scottish population. Patients and methods: A total of 374 CD, 305 ulcerative colitis (UC), and 294 healthy controls (HC) were studied. Ge…

AdultMaleLetterGenotypePopulationNod2 Signaling Adaptor ProteinSingle-nucleotide polymorphismBiologyInflammatory bowel diseaseLoss of heterozygosityCrohn DiseaseGene FrequencyGenotypemedicineHumansGenetic Predisposition to DiseaseeducationGenotypingAllele frequencyGeneticseducation.field_of_studyTumor Suppressor ProteinsInflammatory Bowel DiseaseHaplotypeGastroenterologyIntracellular Signaling Peptides and ProteinsMembrane ProteinsEpistasis GeneticMiddle Agedmedicine.diseaseInflammatory Bowel Diseasesdigestive system diseasesPhenotypeHaplotypesScotlandImmunologyColitis UlcerativeFemale
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No association of alcohol dependence with HOMER 1 and 2 genetic variants.

2010

Several lines of evidence indicate that alterations of the central cortico-accumbens glutamate pathway are involved in the development and maintenance of alcohol- and substance-use disorders. The HOMER protein family is encoded by 3 genes HOMER (1–3) which are components of the excitatory postsynaptic density complex and function to modulate synaptic activity by the regulation of glutamate signaling. HOMER 1 and 2 have been reported to contribute to chronic alcohol-induced long-term neurochemical changes in the endogenous reward system. Data from animal models suggest a potential role of the Homer protein family in the development of alcohol and substance use. The aim of this study is to as…

AdultMaleLinkage disequilibriumSingle-nucleotide polymorphismBiologyPolymorphism Single NucleotideLinkage DisequilibriumCellular and Molecular NeuroscienceGene FrequencyHomer Scaffolding ProteinsGenotypeGenetic variationSNPHumansGenetic Predisposition to DiseaseAlleleGenetics (clinical)GeneticsAlcohol dependenceHaplotypePsychiatry and Mental healthAlcoholismHaplotypesCase-Control StudiesFemaleCarrier ProteinsAmerican journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics
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Genome-wide association data provide further support for an association between 5-HTTLPR and major depressive disorder.

2013

Abstract Background Dysfunctions of serotonergic neurotransmission are supposed to be involved in the pathogenesis of psychiatric disorders such as major depressive disorder (MDD). The concentration of serotonin (5-hydroxytryptamine, 5-HT) in the synaptic cleft is essentially regulated by the 5-HT transporter (5-HTT). A length polymorphism repeat in the 5-HTT promoter region, termed 5-HTTLPR, has been commonly investigated for an association with psychiatric disorders. Methods Genotyping of the 5-HTTLPR is time-consuming and technically challenging. Recently, a two-SNP haplotype was identified that tags the 5-HTTLPR at r 2 =0.775. This allows extraction of 5-HTTLPR genotype information from…

AdultMaleLinkage disequilibriumSynaptic cleftGenotypeSingle-nucleotide polymorphismGenome-wide association studyPolymorphism Single NucleotideGermanygenetics [Haplotypes]mental disordersGenotypegenetics [Serotonin Plasma Membrane Transport Proteins]medicineHumansGenetic Predisposition to Diseaseddc:610GeneticsSerotonin Plasma Membrane Transport ProteinsDepressive Disorder MajorSLC6A4 protein humanHaplotypegenetics [Depressive Disorder Major]Middle Agedmedicine.diseasePsychiatry and Mental healthClinical PsychologyHaplotypes5-HTTLPRCase-Control Studiesgenetics [Polymorphism Single Nucleotide]Major depressive disorderFemalePsychologyClinical psychologyGenome-Wide Association StudyJournal of affective disorders
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HLA-B8,DR3 haplotype affects lymphocyte blood levels

1997

The number of lymphocytes in the blood is constant, pointing to an effective control of circulating lymphocyte values. The mechanisms of this regulation are uncertain, although it is likely that the number of blood lymphocytes is conditioned by hormones, homing factors and cytokines whose production is at least partly restrained by genetic factors. Particularly genetic factors linked to major histocompatibility complex (MHC) appear to be involved. In human beings a decreased number of blood lymphocytes has been described in healthy subjects carrying the Human Leucocyte Antigens (HLA) haplotype HLA-B8,DR3. In the present study, to inquire into the mechanisms of this lymphocyte decreased numb…

AdultMaleLymphocyteImmunologyApoptosisHuman leukocyte antigenMajor histocompatibility complexHLA-B8 AntigenHLA-DR3 AntigenLymphocyte subpopulationsmedicineHumansLymphocyte CountLymphocytesAgedbiologyHaplotypeGeneral MedicineMiddle Agedmedicine.anatomical_structureHaplotypesImmunologybiology.proteinLeucocyte antigensFemaleHormoneHoming (hematopoietic)Immunological Investigations
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Could mitochondrial haplogroups play a role in sporadic amyotrophic lateral sclerosis?

2004

Mitochondrial impairment has been implicated in the pathogenesis of the amyotrophic lateral sclerosis (ALS). Furthermore, mitochondrial-specific polymorphisms were previously related to other neurodegenerative diseases, such as Parkinson, Friedreich and Alzheimer disease. To investigate if specific genetic polymorphisms within the mitochondrial genome (mtDNA) could act as susceptibility factors and contribute to the clinical expression of sporadic ALS (sALS), we have genotyped predefined European mtDNA haplogroups in 222 Italian patients with sALS and 151 matched controls. Individuals classified as haplogroup I demonstrated a significant decrease in risk of ALS versus individuals carrying t…

AdultMaleMitochondrial DNAPathologymedicine.medical_specialtyDiseaseBiologyDNA MitochondrialHaplogroupCohort StudiesDegenerative diseaseConfidence IntervalsOdds RatiomedicineHumansAmyotrophic lateral sclerosisAgedALS; Haplogroups; mtDNA;Polymorphism GeneticmtDNAGeneral NeuroscienceAmyotrophic Lateral SclerosisOdds ratioMiddle Agedmedicine.diseaseMitochondriaALS; mtDNA; HaplogroupsHaplotypesALS; Haplogroups; mtDNAImmunologyHaplogroupsFemaleAlzheimer's diseaseALSHuman mitochondrial DNA haplogroup
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Association of functional DBH genetic variants with alcohol dependence risk and related depression and suicide attempt phenotypes: Results from a lar…

2012

Abstract Objective Dopamine-beta-hydroxylase (DBH) metabolizes the conversion of dopamine to noradrenaline. DBH, located on chromosome 9q34.2 has variants with potential functional consequences which may be related to alterations of neurotransmitter function and several psychiatric phenotypes, including alcohol dependence (AD), depression (MD) and suicidal behavior (SA). The aim of this association study in a large multicenter sample of alcohol-dependent individuals and controls is to investigate the role of DBH SNPs and haplotypes in AD risk and associated phenotypes (AD with MD or SA). Method 1606 inpatient subjects with DSM-IV AD from four addiction treatment centers and 1866 control sub…

AdultMaleOncologymedicine.medical_specialtyGenotypePoison controlSuicide AttemptedSingle-nucleotide polymorphismDopamine beta-HydroxylaseToxicologyPolymorphism Single NucleotideRisk AssessmentLinkage DisequilibriumGermanyInternal medicinemedicineHumansSNPPharmacology (medical)Age of OnsetDepression (differential diagnoses)PharmacologyDepressive DisorderSex CharacteristicsSuicide attemptAlcohol dependenceHaplotypeDNAMiddle AgedAlcoholismPsychiatry and Mental healthPhenotypeCase-Control StudiesSample SizeEtiologyFemalePsychologyGenome-Wide Association StudyClinical psychologyDrug and Alcohol Dependence
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Successful application of preimplantation genetic diagnosis for hypokalaemic periodic paralysis.

2009

Hypokalaemic periodic paralysis is a rare dominant inherited disease where a person suffers sudden falls of circulating potassium concentrations, producing muscle weakness and sometimes severe paralysis. Attacks can occur as frequently as several times a day or once in a year. The age of onset is usually adolescence but symptoms can appear as early as 10 years of age. Muscle weakness can compromise vital functions such as breathing or swallowing and heart arrhythmias are also frequent during attacks. Preimplantation genetic diagnosis, an early form of prenatal diagnosis for couples at risk of transmitting inherited diseases, was used to prevent the transmission of this disease. Six polymorp…

AdultMalePediatricsmedicine.medical_specialtyPathologyHypokalemic Periodic ParalysisPrenatal diagnosisDiseaseBiologyPreimplantation genetic diagnosisPolymerase Chain ReactionHypokalemic periodic paralysismedicineParalysisHumansPreimplantation DiagnosisDNA PrimersBase SequenceHaplotypeObstetrics and GynecologyMuscle weaknessmedicine.diseasePedigreeReproductive MedicineFemaleAge of onsetmedicine.symptomDevelopmental BiologyReproductive biomedicine online
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HLA Class I and Class II Polymorphism in Three Sicilian Populations

2007

Two human leukocyte antigen (HLA) class I loci (HLA-A and HLA-B) and one class II locus (HLA-DR) were typed at the DNA level in the Sicilian population. Study participants were of Sicilian origin (183 for class I loci and 260 for class II loci) and live in three towns, chosen on the basis of geographic position and different historical events. These towns are Sciacca (southwest Sicily, located at sea level, conquered by Arabs in a.d. 814), Piana degli Albanesi (northwest Sicily, 720 m above sea level, has maintained religious, cultural, and linguistic peculiarities traced to Albanian settlement in 1488), and Troina (northeast Sicily, 1,120 m above sea level, known as the first settlement of…

AdultMalePopulationLocus (genetics)Human leukocyte antigenLinkage DisequilibriumGene FrequencyGeneticsHumansAlleleeducationSicilyAllele frequencyAllelesGenetics (clinical)Ecology Evolution Behavior and Systematicseducation.field_of_studyPolymorphism GeneticHLA-A AntigensGenetic VariationHLA-DR Antigenslanguage.human_languageGenetics PopulationHaplotypesHLA-B AntigensChild PreschoolGenetic structurelanguageEthnologyFemalePhoenicianDNA typing haplotype frequency HLA polymorphism sicilian populationSicilianHLA-DRB1 ChainsDemographyHuman Biology
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Oculopharyngeal muscular dystrophy in a northern German family linked to chromosome 14q, and presenting carnitine deficiency

1997

We report the evaluation of oculopharyngeal muscular dystrophy (OPMD) in a large northern German family, which can be traced back six generations and is unrelated to French-Canadian families. The symptoms in this family start at about 50 years of age and include dysphagia, bilateral ptosis, and in some cases a slowly progressive atrophy and weakness of other extraocular, facial or limb girdle muscles. The muscle biopsies showed the pathognomonic ultrastructural finding of characteristic intranuclear filaments. Linkage analysis confirmed that this family is also linked to chromosome 14q markers. Haplotype analysis revealed that a unique haplotype segregates with the disease which is differen…

AdultMaleProbandPathologymedicine.medical_specialtyWeaknessGenetic LinkageBiopsyBiologyMuscular DystrophiesOculopharyngeal muscular dystrophySural NervePathognomonicGenetic linkageCarnitineGermanymedicineHumansCarnitineGenetics (clinical)AgedChromosomes Human Pair 14Family HealthGeneticsElectromyographyHaplotypeMiddle Agedmedicine.diseaseDysphagiaMitochondriaPedigreeMicroscopy ElectronPhenotypeNeurologyOculomotor MusclesPediatrics Perinatology and Child HealthPharyngeal MusclesFemaleNeurology (clinical)medicine.symptommedicine.drugNeuromuscular Disorders
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Variable phenotypic expression of chylomicron retention disease in a kindred carrying a mutation of the Sara2 gene

2010

Chylomicron retention disease is a recessive inherited disorder characterized by fat malabsorption and steatorrhea and is associated with failure to thrive in infancy. We describe a kindred carrying a mutation of Sara2 gene causing a chylomicron retention phenotype. The proband was a 5-month-old baby, born of consanguineous, apparently healthy parents from Morocco, with failure to thrive. There was a large quantity of fats in feces and malabsorption of fat-soluble vitamins. Intestinal biopsies showed a diffused enterocyte vacuolization with large cytosolic lipid droplets. Chylomicron retention disease or Anderson disease was hypothesized, and the Sara2 gene was analyzed by direct sequencing…

AdultMaleProbandmedicine.medical_specialtychylomicron retention disease phenotypic expression Sara2Settore MED/09 - Medicina InternaMalabsorptionEndocrinology Diabetes and MetabolismBiologySettore MED/42 - Igiene Generale E ApplicataExonEndocrinologyMalabsorption SyndromesInternal medicineChylomicronsmedicineHumansAlleleMonomeric GTP-Binding ProteinsGeneticsHaplotypeInfantmedicine.diseaseSteatorrheaPedigreeFat malabsorptionPhenotypeEndocrinologyChild PreschoolMutationFailure to thriveFabry DiseaseFemalemedicine.symptomChylomicron retention diseaseMetabolism
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