Search results for "Stein-"

showing 10 items of 77 documents

Inheritance and variable expression in Rubinstein-Taybi syndrome.

2010

Familial Rubinstein-Taybi syndrome (RTS) is very rare. Here we report on the 6th and 7th case of inherited RTS. Family 1 presents with incomplete or mild RTS over three generations; a 13-year-old girl (proband 1) with mild but typical facial features and learning disabilities, her very mildly affected mother (proband 2), and the maternal grandmother (proband 3). Family 2 includes three females with classical RTS (probands 4-6) and their father (proband 7) with broad thumbs and halluces. Proband 5 also had a brain tumor (ganglioglioma) at the age of 3 years. In probands 1-3, direct sequencing identified a novel CREBBP missense mutation, c.2728A > G (predicting p.Thr910Ala), that was absent i…

ProbandMaleRiskAdolescentDNA Mutational AnalysisMutation MissenseBiologyVariable ExpressionGenetic HeterogeneityGeneticsmedicineMissense mutationHumansPoint MutationFamilyAlleleGenetics (clinical)GeneticsRubinstein-Taybi SyndromeRubinstein–Taybi syndromeGenetic heterogeneityMosaicismPoint mutationmedicine.diseaseCREB-Binding ProteinPedigreePhenotypeChild PreschoolMutation (genetic algorithm)FemaleAmerican journal of medical genetics. Part A
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Analisi MLPA del gene CREB-binding protein (CREBBP) in un paziente con la sindrome di Rubinstein Taybi

2012

La sindrome di Rubinstein-Taybi è una rara malattia congenita autosomica dominante caratterizzata da ritardo della crescita postnatale, ritardo dello sviluppo psicomotorio, anomalie scheletriche, peculiare morfologia facciale ed un aumento del rischio oncogeno. La prevalenza alla nascita è 1 su 125.000 nati vivi. La malattia può essere associata a mutazioni nel gene che codifica per la proteina CREB-binding localizzato nella regione cromosomica 16p13.3. Recenti studi hanno dimostrato che pazienti con quoziente intellettivo basso e tratti autistici possono avere grandi delezioni. Sulla base di queste osservazioni, abbiamo usato la Multiplex Ligation-dependent Probe Amplification (MLPA) per r…

Rubinstein-Taybi Multiplex Ligation-dependent Probe Amplification (MLPA)Settore BIO/13 - Biologia Applicata
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Rubinstein-Taybi syndrome (CREBBP, EP300)

2011

1.2 OMIM# of the disease180849.1.3 Name of the analyzed genes or DNA/chromosome segmentsCREBBP, EP300 (E1A binding protein p300).1.4 OMIM# of the genes600140 (CREBBP), 602700 (EP300).1.5 Mutational spectrumMainly frameshift, nonsense, splice site and missense mutations. Lessfrequently large deletions (one or more exons) and rarely balancedinversions and translocations. Mutations are heterozygous, and mosaicmutations have been described. At present, more than 100 pathogenicmutations are known for the two genes together, but mutations inEP300 are much less common (only 11 so far).

Rubinstein-Taybi SyndromeGeneticsMutationRubinstein–Taybi syndromebiologymedicine.disease_causemedicine.diseaseCREB-Binding ProteinSensitivity and SpecificityMolecular biologyFrameshift mutationExonPredictive Value of TestsMutationClinical Utility Gene CardGeneticsmedicinebiology.proteinHumansMissense mutationCREB-binding proteinEP300E1A-Associated p300 ProteinGeneGenetics (clinical)
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Tissue-specific mosaicism in a patient with Rubinstein–Taybi syndrome and CREBBP exon 1 duplication

2019

Rubinstein-Taybi SyndromeGeneticsRubinstein–Taybi syndromeMosaicismbusiness.industryFaciesExonsGeneral Medicinemedicine.diseaseCREB-Binding ProteinPathology and Forensic MedicineExonOrgan SpecificityGene DuplicationPediatrics Perinatology and Child HealthGene duplicationmedicineHumansTissue specificFemaleAnatomyChildbusinessE1A-Associated p300 ProteinGenetics (clinical)Clinical Dysmorphology
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The Global Side of the Investments-Savings Puzzle

2008

In this paper we re-examine the long standing and puzzling correlation between national savings and investment in industrial countries. We apply an econometric methodology that allows us to separate idiosyncratic correlation at the country level from correlation at the global level. In a major break with the existing literature, we find no evidence of a long run relationship in the idiosyncratic components of savings and investment. We also find that the global components in savings and investments commove, indicating that they react to shocks of a global nature.

Savings Investment Feldstein-Horioka Puzzle Panel Nonstationarity Principal Components.jel:C31jel:F32jel:C33jel:F41
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Coinfection of Helicobacter pylori and Epstein-Barr virus in patients with gastric disease in Southern Italy

Helicobacter pylori

Settore MED/07 - Microbiologia E Microbiologia ClinicaHelicobacter pyloriEpstein-Barr.
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Cross-Commodity Spot Price Modeling with Stochastic Volatility and Leverage For Energy Markets

2013

Spot prices in energy markets exhibit special features, such as price spikes, mean reversion, stochastic volatility, inverse leverage effect, and dependencies between the commodities. In this paper a multivariate stochastic volatility model is introduced which captures these features. The second-order structure and stationarity of the model are analyzed in detail. A simulation method for Monte Carlo generation of price paths is introduced and a numerical example is presented.

Statistics and Probability15A04Spot contractSABR volatility model01 natural sciences010104 statistics & probabilityEnergy marketVolatility swap0502 economics and businessEconometricsForward volatilityMean reversionstochastic volatilityleverage0101 mathematicsMathematics050208 financeStochastic volatilityApplied Mathematics05 social sciences91G60subordinator91G20Constant elasticity of variance modelVolatility smileOrnstein-Uhlenbeck process60H3060G1060G51Advances in Applied Probability
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Einstein-Smoluchowsky equation handled by complex fractional moments

2014

In this paper the response of a non linear half oscillator driven by α-stable white noise in terms of probability density function (PDF) is investigated. The evolution of the PDF of such a system is ruled by the so called Einstein-Smoluchowsky equation involving, in the diffusive term, the Riesz fractional derivative. The solution is obtained by the use of complex fractional moments of the PDF, calculated with the aid of Mellin transform operator. It is shown that solution can be found for various values of stability index α and for any nonlinear function of the drift term in the stochastic differential equation.

Stochastic partial differential equationNonlinear systemStochastic differential equationMellin transformDifferential equationOperator (physics)Mathematical analysisProbability density functiona-stable white noise Nonlinear systems Einstein-Smoluchowsky equation Complex fractional momentsFractional calculusMathematics
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Noise contribution to resonance phenomena and information propagation in non linear electronic networks

2015

This manuscript presents research aiming to show possible positive effects of deterministic and stochastic perturbations on the responses of different nonlinear systems. To that end, both numerical and experimental studies were carried out on two kinds of structures : an elementary electronic FitzHugh-Nagumo oscillator and an electrical line developed by resistively coupling 45 elementary cells. In the first section, the elementary cell characterization was undertaken in a deterministic regime. In the presence of a bichromatic stimulus, it is shown that when the low frequency component is subthreshold, its detection can be maximized for an optimal magnitude of the second component thanks to…

Vibrational resonanceGhost stochastic resonanceFrequency resonanceRésonance fréquentielleDynamique non linéaireDeterministic perturbationProcessus d’Ornstein-UhlenbeckVibrational propagationPerturbation déterministeElectronic circuitWhite noiseCircuit électroniqueColored noisePropagation vibrationnelle[SPI.TRON] Engineering Sciences [physics]/Electronics[SPI.TRON]Engineering Sciences [physics]/Electronics[ SPI.TRON ] Engineering Sciences [physics]/ElectronicsRésonance vibrationnellePropagation assistée par le bruitNonlinear dynamicsBruit coloréOrnstein-Uhlenbeck processBruit blancNoise assisted propagationRésonance stochastique fantômeFitzHugh-Nagumo
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PARAMETER ESTIMATION FOR FRACTIONAL ORNSTEIN-UHLENBECK PROCESSES: NON-ERGODIC CASE

2011

We consider the parameter estimation problem for the non-ergodic fractional Ornstein-Uhlenbeck process defined as $dX_t=\theta X_tdt+dB_t,\ t\geq0$, with a parameter $\theta>0$, where $B$ is a fractional Brownian motion of Hurst index $H\in(1/2,1)$. We study the consistency and the asymptotic distributions of the least squares estimator $\hat{\theta}_t$ of $\theta$ based on the observation $\{X_s,\ s\in[0,t]\}$ as $t\rightarrow\infty$.

[MATH.MATH-PR] Mathematics [math]/Probability [math.PR]Probability (math.PR)62F12 60G18 60G1562F12 60G18 60G15.[MATH.MATH-PR]Mathematics [math]/Probability [math.PR]Mathematics::ProbabilityFOS: MathematicsParameter estimationYoung integralYoung integral.Parameter estimation; Non-ergodic fractional Ornstein-Uhlenbeck process; Young integral.[ MATH.MATH-PR ] Mathematics [math]/Probability [math.PR]Mathematics - ProbabilityNon-ergodic fractional Ornstein-Uhlenbeck process
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