Williams syndrome (WMS) is a rare sporadic disorder that yields a distinctive profile of medical, cognitive, neurophysiological, neuroanatomical and genetic characteristics. The cognitive hallmark of WMS is a dissociation between language and face processing (relative strengths) and spatial cognition (profound impairment). Individuals with WMS also tend to be overly social, behavior that is opp...

Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. A critical component of such a cross-domain ...

Williams syndrome (WMS) is a most compelling model of human cognition, of human genome organization, and of evolution. Due to a deletion in chromosome band 7q11.23, subjects have cardiovascular, connective tissue, and neurodevelopmental deficits. Given the striking peaks and valleys in neurocognition including deficits in visual-spatial and global processing, preserved language and face process...

BACKGROUND Diets high in either resistant starch or protein have been shown to aid in weight management. We examined the effects of meals high in non-resistant or resistant starch with and without elevated protein intake on substrate utilization, energy expenditure, and satiety in lean and overweight/obese women. METHODS Women of varying levels of adiposity consumed one of four pancake test m...

The rare, genetically based disorder, Williams syndrome (WMS), produces a constellation of distinctive cognitive, neuroanatomical, and electrophysiological features which we explore through the series of studies reported here. In this paper, we focus primarily on the cognitive characteristics of WMS and begin to forge links among these characteristics, the brain, and the genetic basis of the di...

In recent years the sophistication and application of Warehouse Management Systems (WMS) has increased significantly and they are now seen as vital systems for gaining efficiency improvements and cost savings within the logistics industry. However, the successful implementation of a WMS is rare. Therefore, this study’s aims were to explore the key factors that influence the success of a WMS dev...

Williams syndrome (WMS), a rare neurogenetic disorder, has been in the forefront of research in cognitive psychology for the last ten years. WMS is characterized by a distinctive cognitive pro le: mild to moderate mental retardation with relatively and surprisingly good linguistic abilities, while performance on spatial tasks is extremely poor. Concentrating on the linguistic abilities of child...

Individuals with Williams syndrome (WMS) show an interesting dissociation of ability within the visuospatial domain, particularly between face perception and other visuospatial tasks. In this population, using tasks matched for stimuli, required response, and difficulty (for controls) is critical when comparing performance across these areas. We compared WMS individuals with a sample of typical...

Use of the common marmoset (Callithrix jacchus) as a non-human primate experimental animal has increased in recent years. Although wasting marmoset syndrome (WMS) is one of the biggest problems in captive marmoset colonies, the molecular mechanisms, biochemical markers for accurate diagnosis and a reliable treatment remain unknown. In this study, as a first step to finding biochemical marker(s)...

A mid-infrared absorption strategy with calibration-free wavelength-modulation-spectroscopy (WMS) has been developed and demonstrated for real-time, in situ detection of nitric oxide in particulate-laden combustionexhaust gases up to temperatures of 700 K. An externalcavity quantum-cascade laser (ECQCL) near 5.2 μm accessed the fundamental absorption band of NO, and a wavelength-scanned, 1f -no...