The name of lysosomal storage diseases stems from the fact that in this category of disorders specific undegraded materials are stored in the lysosomes. This is usually caused by a lysosomal enzyme deficiency and leads to a cascade of pathological outcomes. Apart from deficiency of lysosomal enzymes, lysosomal storage diseases also include deficiencies in proteins necessary for enzyme functioni...

This study developed a series of strategies to reassess the service level winter road maintenance (WRM) operations on network in Perth County, Ontario, Canada. These include rearranging plow routes by sharing material storage yards and optimizing their numbers locations while considering operational legislative constraints such as total circuit time, operating speeds, number available trucks. A...

This document describes a file format for the storage of flow data based upon the IP Flow Information Export (IPFIX) protocol. It proposes a set of requirements for flat-file, binary flow data file formats, then specifies the IPFIX File format to meet these requirements based upon IPFIX Messages. This IPFIX File format is designed to facilitate interoperability and reusability among a wide vari...

Grain and fertiliser transportation are the fastest growing segments of services market, including railway transportation. Presidential Decree No. 204 7 May 2018 “On national goals strategic objectives for development Russian Federation period until 2024” tasked government with developing a programme measures to increase productivity economic sectors, agroindustrial complex. The in latter shoul...

during recent years oil products has decreased because of growing population and increasing demand to oil products and decreasing of crude oil extraction at compare to export. however, to achieve suitable levels of production, the use of improved technologies should be considered. another way to prevent the evaporation and leakage from oil storage tanks and their products are using of storage t...

This study investigated glucocorticoid (GC) responses to season and changes in enclosure size and human proximity in the Persian onager (Equus hemionus onager). Enzyme immunoassays were validated to measure GC metabolites in urine and feces (fGCM). Fecal samples were collected from 10 female onagers while in a large pasture, after transport to smaller yards (in greater proximity to people), and...

[1] Arctic Ocean freshwater budgets are examined from 10 models participating in the Intergovernmental Panel on Climate Change Fourth Assessment Report. This includes an analysis of sea ice transport and storage, ocean transport and storage, and net surface flux exchange. Simulated budgets for the late 20th century are compared to available observations, followed by an analysis of simulated cha...