No abstract

XLH in humans and the Hyp phenotype in mice are caused by inactivating Phex mutations. Overexpression of human PHEX under the human ␤-actin promoter in Hyp mice rescued the bone phenotype almost completely, but did not affect phosphate homeostasis, suggesting that different, possibly independent, pathophysiological mechanisms contribute to hyperphosphaturia and bone abnormalities in XLH.

No abstract

In light of artifact‐induced high variability of activation in fMRI repeat studies, we developed and tested a clinically useful plaster cast head holder (PCH) with improved immobilization, repositioning, and comfort. With PCH, there were considerably lower levels of translational and rotational head motion components compared to head fixation with conventional restraining straps (CRS). Rotational components cannot be fully compensated by realignment and lead to “false activations.” In addition, task‐correlated head motion, which highly increases the risk of artifacts, was considerably reduced with PCH, especially in a motion prone subject. Compared with PCH, head motion was 133% larger with CRS in a highly cooperative subject. With a motion prone subject, head motion range was increased by 769% (PCH: 0.9 mm, CRS: 7.8 mm), which may indicate the usefulness of PCH for restless patients. In functional activation maps, PCH alone yielded fewer residual motion artifacts than CRS + image registration. Subject tolerance of the head holder during the long measurement times of up to 2.5 hr was good, and slice orientation on different days confirmed the quality of repositioning. Hum. Brain Mapping 11:207–213, 2000. © 2000 Wiley‐Liss, Inc.

We study the probability for snow cover at a climate station. Connecting stations with the same probability yields the corresponding snow line (a figure between zero and unity). The climatological snow lines in the Alps are implicit in the state function of snow duration. This function, specified by just five parameters, depends upon the mountain temperature, a linear combination of the mean temperature over Europe and the 3D-coordinates of the stations. The influence of external parameters other than temperature (like snowfall, melting processes, station exposition) is treated as stochastic. The five state function parameters are gained for both winter (DJF) and summer (JJA) through a fitting algorithm from routine snow depth observations in 1961-2010 in Austria and Switzerland. Any desired snow line is defined by a linear surface with a characteristic value of the mountain temperature. The snow line appears when there is a cut between the surface and the orography. Temperature sensitivity of snow cover duration, analytically derived from the state function, is extreme at the median snow line (snow probability 0.50). Alpine-wide mean altitude of the median snow line is 793(±36)m in winter and 3.083(±1.121)m in summer. The snowline field slopes gently from west to east across the Alps (downward in winter, upward in summer) and oscillates up and down with the sea