Background: Cystinosis is a rare autosomal recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. It is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. Elevated white blood cell cystine content is the cornerstone of the diagnosis. Since chitotriosidase (CHIT1 or chitinase-1) is mainly produced by activated macrophages both in normal and inflammator

The importance of this study lies in shedding the light on the impact of Islam and Prophet Mohammed (peace be upon him) on the western culture and English literature in particular. While some writers were looking at Islam as a dangerous element, others were completely taken by the oriental spirit of Arabic and Islamic culture and glorifying it. Writers from Chaucer to later ones mostly make references to this impact showing how vast was the gap of misunderstanding between the east and the West. Thus, this study aims at breaking the barrier between East and West in its three sections as it introduces the meaning of Islam and its common features with other religions in the first section. The second section briefly presents writers’ reflecti

This study aims to find out the effect of the mediator on scaffolding fourth yearstudent- teachers' teaching competencies and their self-efficacy. The present study combines scaffolding and self-efficacy by using a mediator on scaffolding students affects teaching competencies and selfefficacy and from the results of which the existence of student-teachers’ selfawareness was ensured as an effect of the same independent variable. The model affects their teaching competencies and led them to be aware of the needs of their pupils and themselves. 

Transgenic plants offer advantages for the manufacture of recombinant proteins with terminal
mannose residues on their glycan chains. So plants are chosen as source of pharmaceutical products and for
the development of alternative expression systems to produce recombinant lysosomal enzymes. In the
present study the sequence of the natural cDNA encoding for the human lysosomal enzyme
glucocerebrosidase (GCD) was modified to enhance its expression in soybean plants. The glucocerebrosidase
gene signal peptide was substituted with that signal peptide for the Arabidopsis thaliana basic endochitinase
gene to support the co-translational translocation into the endoplasmic reticulum (ER), and the storage
vacuol

The primary goal of root canal treatment (RCT) is to expel the presence of any necrotic or vital tissue, microbes and their byproducts from the canal space before press forward with the following steps of the RCT procedures. Although this is difficult to attain, various strives had been practiced by employing chemical and mechanical methods to eliminate as much microorganisms as possible and make the canal space valid for the obturation materials to be received. The aim of this review is to demonstrate some of what new remedies that could be used as root canal disinfectant by summarizing the recent studies regarding the efficacy of different natural products against the most persistence microbiota that could be responsible for most

Low-temperature stratification, high-volumetric storage capacity, and less-complicated material processing make phase-changing materials (PCMs) very suitable candidates for solar energy storage applications. However, their poor heat diffusivities and suboptimal containment designs severely limit their decent storage capabilities. In these systems, the arrangement of tubes conveying the heat transport fluid (HTF) plays a crucial role in heat communication between the PCM and HTF during phase transition. This study investigates a helical coil tube-and-shell thermal storage system integrated with a novel central return tube to enhance heat transfer effectiveness. Three-dimensional computational fluid dynamics simulations compare the proposed d

Separation of Trigonelline, the major alkaloid in fenugreek seeds, is difficult because the extract of these seeds usually contains Trigonelline, choline, mucilage, and steroidal saponins, in addition to some other substances. This study amis to isolate the quaternary ammonium alkaloid (Trigonelline) and choline from fenugreek seeds (Trigonella-foenum graecum L.) which have similar physiochemical properties by modifying of the classical method. Seeds were defatted and then extracted with methanol. The presence of alkaloids was detected by using Mayer's and Dragendorff's reagents. In this work, trigonilline was isolated with traces of choline by subsequent processes of purification using analytical and preparative TLC techniques.