Mutations in the TGFβ binding-protein-like domain 5 of FBN1 are responsible for acromicric and geleophysic dysplasias.

Geleophysic (GD) and acromicric dysplasia (AD) belong to the acromelic dysplasia group and are both characterized by severe short stature, short extremities, and stiff joints. Although AD has an unknown molecular basis, we have previously identified ADAMTSL2 mutations in a subset of GD patients. After exome sequencing in GD and AD cases, we selected fibrillin 1 (FBN1) as a candidate gene, even though mutations in this gene have been described in Marfan syndrome, which is characterized by tall stature and arachnodactyly. We identified 16 heterozygous FBN1 mutations that are all located in exons 41 and 42 and encode TGFβ-binding protein-like domain 5 (TB5) of FBN1 in 29 GD and AD cases. Microfibrillar network disorganization and enhanced TGFβ signaling were consistent features in GD and AD fibroblasts. Importantly, a direct interaction between ADAMTSL2 and FBN1 was demonstrated, suggesting a disruption of this interaction as the underlying mechanism of GD and AD phenotypes. Although enhanced TGFβ signaling caused by FBN1 mutations can trigger either Marfan syndrome or GD and AD, our findings support the fact that TB5 mutations in FBN1 are responsible for short stature phenotypes.

High capacity audio watermarking using the high frequency band of the wavelet domain

This paper proposes a novel high capacity robust audio watermarking algorithm by using the high frequency band of the wavelet decomposition at which the human auditory system (HAS) is not very sensitive to alteration. The main idea is to divide the high frequency band into frames and, for embedding, to change the wavelet samples depending on the average of relevant frame¿s samples. The experimental results show that the method has a very high capacity (about 11,000 bps), without significant perceptual distortion (ODG in [¿1 ,0] and SNR about 30dB), and provides robustness against common audio signal processing such as additive noise, filtering, echo and MPEG compression (MP3).

Jagged1 intracellular domain-mediated inhibition of Notch1 signalling regulates cardiac homeostasis in the postnatal heart.

AIMS: Notch1 signalling in the heart is mainly activated via expression of Jagged1 on the surface of cardiomyocytes. Notch controls cardiomyocyte proliferation and differentiation in the developing heart and regulates cardiac remodelling in the stressed adult heart. Besides canonical Notch receptor activation in signal-receiving cells, Notch ligands can also activate Notch receptor-independent responses in signal-sending cells via release of their intracellular domain. We evaluated therefore the importance of Jagged1 (J1) intracellular domain (ICD)-mediated pathways in the postnatal heart. METHODS AND RESULTS: In cardiomyocytes, Jagged1 releases J1ICD, which then translocates into the nucleus and down-regulates Notch transcriptional activity. To study the importance of J1ICD in cardiac homeostasis, we generated transgenic mice expressing a tamoxifen-inducible form of J1ICD, specifically in cardiomyocytes. Using this model, we demonstrate that J1ICD-mediated Notch inhibition diminishes proliferation in the neonatal cardiomyocyte population and promotes maturation. In the neonatal heart, a response via Wnt and Akt pathway activation is elicited as an attempt to compensate for the deficit in cardiomyocyte number resulting from J1ICD activation. In the stressed adult heart, J1ICD activation results in a dramatic reduction of the number of Notch signalling cardiomyocytes, blunts the hypertrophic response, and reduces the number of apoptotic cardiomyocytes. Consistently, this occurs concomitantly with a significant down-regulation of the phosphorylation of the Akt effectors ribosomal S6 protein (S6) and eukaryotic initiation factor 4E binding protein1 (4EBP1) controlling protein synthesis. CONCLUSIONS: Altogether, these data demonstrate the importance of J1ICD in the modulation of physiological and pathological hypertrophy, and reveal the existence of a novel pathway regulating cardiac homeostasis.

Boundary identification in the domain of a parabolic partial differential equation

Bakgrunden och inspirationen till föreliggande studie är tidigare forskning i tillämpningar på randidentifiering i metallindustrin. Effektiv randidentifiering möjliggör mindre säkerhetsmarginaler och längre serviceintervall för apparaturen i industriella högtemperaturprocesser, utan ökad risk för materielhaverier. I idealfallet vore en metod för randidentifiering baserad på uppföljning av någon indirekt variabel som kan mätas rutinmässigt eller till en ringa kostnad. En dylik variabel för smältugnar är temperaturen i olika positioner i väggen. Denna kan utnyttjas som insignal till en randidentifieringsmetod för att övervaka ugnens väggtjocklek. Vi ger en bakgrund och motivering till valet av den geometriskt endimensionella dynamiska modellen för randidentifiering, som diskuteras i arbetets senare del, framom en flerdimensionell geometrisk beskrivning. I de aktuella industriella tillämpningarna är dynamiken samt fördelarna med en enkel modellstruktur viktigare än exakt geometrisk beskrivning. Lösningsmetoder för den s.k. sidledes värmeledningsekvationen har många saker gemensamt med randidentifiering. Därför studerar vi egenskaper hos lösningarna till denna ekvation, inverkan av mätfel och något som brukar kallas förorening av mätbrus, regularisering och allmännare följder av icke-välställdheten hos sidledes värmeledningsekvationen. Vi studerar en uppsättning av tre olika metoder för randidentifiering, av vilka de två första är utvecklade från en strikt matematisk och den tredje från en mera tillämpad utgångspunkt. Metoderna har olika egenskaper med specifika fördelar och nackdelar. De rent matematiskt baserade metoderna karakteriseras av god noggrannhet och låg numerisk kostnad, dock till priset av låg flexibilitet i formuleringen av den modellbeskrivande partiella differentialekvationen. Den tredje, mera tillämpade, metoden kännetecknas av en sämre noggrannhet förorsakad av en högre grad av icke-välställdhet hos den mera flexibla modellen. För denna gjordes även en ansats till feluppskattning, som senare kunde observeras överensstämma med praktiska beräkningar med metoden. Studien kan anses vara en god startpunkt och matematisk bas för utveckling av industriella tillämpningar av randidentifiering, speciellt mot hantering av olinjära och diskontinuerliga materialegenskaper och plötsliga förändringar orsakade av “nedfallande” väggmaterial. Med de behandlade metoderna förefaller det möjligt att uppnå en robust, snabb och tillräckligt noggrann metod av begränsad komplexitet för randidentifiering.

Impact of Word-of-Mouth on consumer decision-making: An information processing perspective in the context of a high-involvement service

This doctoral study conducts an empirical analysis of the impact of Word-of-Mouth (WOM) on marketing-relevant outcomes such as attitudes and consumer choice, during a high-involvement and complex service decision. Due to its importance to decisionmaking, WOM has attracted interest from academia and practitioners for decades. Consumers are known to discuss products and services with one another. These discussions help consumers to form an evaluative opinion, as WOM reduces perceived risk, simplifies complexity, and increases the confidence of consumers in decisionmaking. These discussions are also highly impactful as WOM is a trustworthy source of information, since it is independent from the company or brand. In responding to the calls for more research on what happens after WOM information is received, and how it affects marketing-relevant outcomes, this dissertation extends prior WOM literature by investigating how consumers process information in a highinvolvement service domain, in particular higher-education. Further, the dissertation studies how the form of WOM influences consumer choice. The research contributes to WOM and services marketing literature by developing and empirically testing a framework for information processing and studying the long-term effects of WOM. The results of the dissertation are presented in five research publications. The publications are based on longitudinal data. The research leads to the development of a proposed theoretical framework for the processing of WOM, based on theories from social psychology. The framework is specifically focused on service decisions, as it takes into account evaluation difficulty through the complex nature of choice criteria associated with service purchase decisions. Further, other gaps in current WOM literature are taken into account by, for example, examining how the source of WOM and service values affects the processing mechanism. The research also provides implications for managers aiming to trigger favorable WOM through marketing efforts, such as advertising and testimonials. The results provide suggestions on how to design these marketing efforts by taking into account the mechanism through which information is processed, or the form of social influence.

Investigations On The Development Of An Ann Model & Visual Manipulation Approach For 2-D Dft Computation In Image Processing

This thesis is an outcome of the investigations carried out on the development of an Artificial Neural Network (ANN) model to implement 2-D DFT at high speed. A new definition of 2-D DFT relation is presented. This new definition enables DFT computation organized in stages involving only real addition except at the final stage of computation. The number of stages is always fixed at 4. Two different strategies are proposed. 1) A visual representation of 2-D DFT coefficients. 2) A neural network approach. The visual representation scheme can be used to compute, analyze and manipulate 2D signals such as images in the frequency domain in terms of symbols derived from 2x2 DFT. This, in turn, can be represented in terms of real data. This approach can help analyze signals in the frequency domain even without computing the DFT coefficients. A hierarchical neural network model is developed to implement 2-D DFT. Presently, this model is capable of implementing 2-D DFT for a particular order N such that ((N))4 = 2. The model can be developed into one that can implement the 2-D DFT for any order N upto a set maximum limited by the hardware constraints. The reported method shows a potential in implementing the 2-D DF T in hardware as a VLSI / ASIC

Fast Fractal Coding Method for the Detection of Microcalcification in Mammograms

The presence of microcalcifications in mammograms can be considered as an early indication of breast cancer. A fastfractal block coding method to model the mammograms fordetecting the presence of microcalcifications is presented in this paper. The conventional fractal image coding method takes enormous amount of time during the fractal block encoding.procedure. In the proposed method, the image is divided intoshade and non shade blocks based on the dynamic range, andonly non shade blocks are encoded using the fractal encodingtechnique. Since the number of image blocks is considerablyreduced in the matching domain search pool, a saving of97.996% of the encoding time is obtained as compared to theconventional fractal coding method, for modeling mammograms.The above developed mammograms are used for detectingmicrocalcifications and a diagnostic efficiency of 85.7% isobtained for the 28 mammograms used.

Signal processing of microstructure profiles: integrating turbulent spatial scales in aquatic ecological modeling

The main objective of this thesis was the integration of microstructure information in synoptic descriptors of turbulence, that reflects the mixing processes. Turbulent patches are intermittent in space and time, but they represent the dominant process for mixing. In this work, the properties of turbulent patches were considered the potential input for integrating the physical microscale measurements. The development of a method for integrating the properties of the turbulent patches required solving three main questions: a) how can we detect the turbulent patches from he microstructure measurements?; b) which are the most relevant properties of the turbulent patches?; and ) once an interval of time has been selected, what kind of synoptic parameters could better reflect the occurrence and properties of the turbulent patches? The answers to these questions were the final specific objectives of this thesis.

Compensatory signal processing for profoundly hearing-impaired listeners: the transmission of fundamental frequency

This paper reviews a study to determine the usefulness of signal processing along with lipreading in improving speech perception of profoundly hearing impaired persons.

The neural processing of masked speech: Evidence for different mechanisms in the left and right temporal lobes

It has been previously demonstrated that extensive activation in the dorsolateral temporal lobes associated with masking a speech target with a speech masker, consistent with the hypothesis that competition for central auditory processes is an important factor in informational masking. Here, masking from speech and two additional maskers derived from the original speech were investigated. One of these is spectrally rotated speech, which is unintelligible and has a similar (inverted) spectrotemporal profile to speech. The authors also controlled for the possibility of “glimpsing” of the target signal during modulated masking sounds by using speech-modulated noise as a masker in a baseline condition. Functional imaging results reveal that masking speech with speech leads to bilateral superior temporal gyrus (STG) activation relative to a speech-in-noise baseline, while masking speech with spectrally rotated speech leads solely to right STG activation relative to the baseline. This result is discussed in terms of hemispheric asymmetries for speech perception, and interpreted as showing that masking effects can arise through two parallel neural systems, in the left and right temporal lobes. This has implications for the competition for resources caused by speech and rotated speech maskers, and may illuminate some of the mechanisms involved in informational masking.

The neural processing of masked speech: evidence for different mechanisms in the left and right temporal lobes

It has been previously demonstrated that extensive activation in the dorsolateral temporal lobes associated with masking a speech target with a speech masker, consistent with the hypothesis that competition for central auditory processes is an important factor in informational masking. Here, masking from speech and two additional maskers derived from the original speech were investigated. One of these is spectrally rotated speech, which is unintelligible and has a similar (inverted) spectrotemporal profile to speech. The authors also controlled for the possibility of "glimpsing" of the target signal during modulated masking sounds by using speech-modulated noise as a masker in a baseline condition. Functional imaging results reveal that masking speech with speech leads to bilateral superior temporal gyrus (STG) activation relative to a speech-in-noise baseline, while masking speech with spectrally rotated speech leads solely to right STG activation relative to the baseline. This result is discussed in terms of hemispheric asymmetries for speech perception, and interpreted as showing that masking effects can arise through two parallel neural systems, in the left and right temporal lobes. This has implications for the competition for resources caused by speech and rotated speech maskers, and may illuminate some of the mechanisms involved in informational masking.

High signal to noise ratio THz spectroscopy with ASOPS and signal processing schemes for mapping and controlling molecular and bulk relaxation processes

Asynchronous Optical Sampling has the potential to improve signal to noise ratio in THz transient sperctrometry. The design of an inexpensive control scheme for synchronising two femtosecond pulse frequency comb generators at an offset frequency of 20 kHz is discussed. The suitability of a range of signal processing schemes adopted from the Systems Identification and Control Theory community for further processing recorded THz transients in the time and frequency domain are outlined. Finally, possibilities for femtosecond pulse shaping using genetic algorithms are mentioned.

The application of Prony’s method to time-domain waveform data modeling in the presence of noise

In this paper,the Prony's method is applied to the time-domain waveform data modelling in the presence of noise.The following three problems encountered in this work are studied:(1)determination of the order of waveform;(2)de-termination of numbers of multiple roots;(3)determination of the residues.The methods of solving these problems are given and simulated on the computer.Finally,an output pulse of model PG-10N signal generator and the distorted waveform obtained by transmitting the pulse above mentioned through a piece of coaxial cable are modelled,and satisfactory results are obtained.So the effectiveness of Prony's method in waveform data modelling in the presence of noise is confirmed.

Use of the LMS adaptive signal processing technique to improve signal data rates from practical oil well logging equipment

The simulation and development work that has been undertaken to produce a signal equaliser used to improve the data rates from oil well logging instruments is presented. The instruments are lowered into the drill bore hole suspended by a cable which has poor electrical characteristics. The equaliser described in the paper corrects for the distortions that occur from the cable (dispersion and attenuation) with the result that the instrument can send data at 100 K.bits/second down its own suspension cable of 12 Km in length. The use of simulation techniques and tools were invaluable in generating a model for the distortions and proved to be a useful tool when site testing was not available.

The effects of explicit versus parameterized convection on the MJO in a large-domain high-resolution tropical case study. Part I: Characterization of large-scale organization and propagation

High-resolution simulations over a large tropical domain (∼20◦S–20◦N and 42◦E–180◦E) using both explicit and parameterized convection are analyzed and compared to observations during a 10-day case study of an active Madden-Julian Oscillation (MJO) event. The parameterized convection model simulations at both 40 km and 12 km grid spacing have a very weak MJO signal and little eastward propagation. A 4 km explicit convection simulation using Smagorinsky subgrid mixing in the vertical and horizontal dimensions exhibits the best MJO strength and propagation speed. 12 km explicit convection simulations also perform much better than the 12 km parameterized convection run, suggesting that the convection scheme, rather than horizontal resolution, is key for these MJO simulations. Interestingly, a 4 km explicit convection simulation using the conventional boundary layer scheme for vertical subgrid mixing (but still using Smagorinsky horizontal mixing) completely loses the large-scale MJO organization, showing that relatively high resolution with explicit convection does not guarantee a good MJO simulation. Models with a good MJO representation have a more realistic relationship between lower-free-tropospheric moisture and precipitation, supporting the idea that moisture-convection feedback is a key process for MJO propagation. There is also increased generation of available potential energy and conversion of that energy into kinetic energy in models with a more realistic MJO, which is related to larger zonal variance in convective heating and vertical velocity, larger zonal temperature variance around 200 hPa, and larger correlations between temperature and ascent (and between temperature and diabatic heating) between 500–400 hPa.