Calibration of numercal models for small debris flows in Yosemite Valley, California, USA

This study compares documented debris flow runout distances with numerical simulations in the Yosemite Valley of California, USA, where about 15% of historical events of slope instability can be classified as debris flows and debris slides (Wieczorek and Snyder, 2004). </p><p style='line-height: 20px;'> To model debris flows in the Yosemite Valley, we selected six streams with evidence of historical debris flows; three of the debris flow deposits have single channels, and the other three split their pattern in the fan area into two or more channels. From field observations all of the debris flows involved coarse material, with only very small clay content. </p><p style='line-height: 20px;'> We applied the one dimensional DAN (Dynamic ANalysis) model (Hungr, 1995) and the two-dimensional FLO-2D model (O'Brien et al., 1993) to predict and compare the runout distance and the velocity of the debris flows observed in the study area. As a first step, we calibrated the parameters for the two softwares through the back analysis of three debris- flows channels using a trial-and-error procedure starting with values suggested in the literature. In the second step we applied the selected values to the other channels, in order to evaluate their predictive capabilities. </p><p style='line-height: 20px;'> After parameter calibration using three debris flows we obtained results similar to field observations We also obtained a good agreement between the two models for velocities. Both models are strongly influenced by topography: we used the 30 m cell size DTM available for the study area, that is probably not accurate enough for a highly detailed analysis, but it can be sufficient for a first screening

GIS-based modelling of rock-ice avalanches from Alpine permafrost areas

Changing permafrost conditions caused by present atmospheric warming are expected to affect the stability of steep rock walls in high mountain areas. The possible increase in periglacial slope instabilities and the especially long potential run-out distances in glacial environments require more awareness about the kind of events as well as robust models to foresee areas affected and distances reached. A geographic information system-based flow-routing model is introduced for modelling rock-ice avalanches on a regional scale. The model application to three major historical events in the European Alps shows the basic use for simulating such events for first-order assessments. By designating the path of steepest descent while allowing lateral spreading from the fall track up to 45°, general flow patterns as well as changes in the direction of progression are well reproduced. The run-out distances are determined using empirically based models and suit well the case studies presented

Transformation and americanization : the American turners and their new identity

OBJECTIVE: Short-chain enoyl-CoA hydratase (ECHS1) is a multifunctional mitochondrial matrix enzyme that is involved in the oxidation of fatty acids and essential amino acids such as valine. Here, we describe the broad phenotypic spectrum and pathobiochemistry of individuals with autosomal-recessive ECHS1 deficiency. METHODS: Using exome sequencing, we identified ten unrelated individuals carrying compound heterozygous or homozygous mutations in ECHS1. Functional investigations in patient-derived fibroblast cell lines included immunoblotting, enzyme activity measurement, and a palmitate loading assay. RESULTS: Patients showed a heterogeneous phenotype with disease onset in the first year of life and course ranging from neonatal death to survival into adulthood. The most prominent clinical features were encephalopathy (10/10), deafness (9/9), epilepsy (6/9), optic atrophy (6/10), and cardiomyopathy (4/10). Serum lactate was elevated and brain magnetic resonance imaging showed white matter changes or a Leigh-like pattern resembling disorders of mitochondrial energy metabolism. Analysis of patients&#39; fibroblast cell lines (6/10) provided further evidence for the pathogenicity of the respective mutations by showing reduced ECHS1 protein levels and reduced 2-enoyl-CoA hydratase activity. While serum acylcarnitine profiles were largely normal, in&nbsp;vitro palmitate loading of patient fibroblasts revealed increased butyrylcarnitine, unmasking the functional defect in mitochondrial &beta;-oxidation of short-chain fatty acids. Urinary excretion of 2-methyl-2,3-dihydroxybutyrate - a potential derivative of acryloyl-CoA in the valine catabolic pathway - was significantly increased, indicating impaired valine oxidation. INTERPRETATION: In conclusion, we define the phenotypic spectrum of a new syndrome caused by ECHS1 deficiency. We speculate that both the &beta;-oxidation defect and the block in l-valine metabolism, with accumulation of toxic methacrylyl-CoA and acryloyl-CoA, contribute to the disorder that may be amenable to metabolic treatment approaches