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 problem of research was the lack of research that dealt with issue of the job design approach that is more suitable for knowledge work, therefore, the research aims to determine the impact of job enrichment,  and knowledge capitalon strategic success, starting from the hypothesis that there significant impact of job enrichment and knowledge capital on strategic success, to achieve this goal the researchers from the theoretical literature and  related studies conclude to the construction of the scheme shows the hypothetical relationship between the variables, which was adopted job enrichment as independent variable while knowledge capital plays two roles, the first as an independent variable and the second as an intermediate

This study was carried out to investigate the possibility of chickpea soaked water as a substitute for yeast in dough fermentation and its effects on sensory properties of the laboratory loaf bread. Chickpea was soaked for 24,48 and 72 hours at room temperature and used in proportion with or without yeast in dough fermentation . The results revealed that , as the percentage of soaked chickpea water substitution increased, the volume of the produced loaf bread decreased as compared with the control treatment (only yeast ).Best results were obtained by using soaked chickpea water for 24 hours in proportion of 1:1 soaked chickpea water : yeast regarding the sensory properties ,volume and leavening of the loaf bread.
Keywords: chickpea so

إن النجاح في أداء المتطلبات الفنية والخططية في أي من الألعاب ألرياضيه يستوجب امتلاك العناصر الاساسيه المتعلقة بطبيعة الاداء ونوع الفعالية الرياضية الممارسة , لذا فان اغلب الألعاب الرياضية تعتمد على مكونات ألقدره التوافقيه والادراكيه الحسيه بوصفها احد العناصر الاساسيه في المستويات العليا لما توفره من قاعدة اقتران للصفات البدنيه والحر كيه وقدرات أجهزة الجسم الوظيفية , وفقا للأسس المعتمدة في بناء مهاراته, وع

In this work, the effect of vortex shedding on the solar collector performance of the parabolic trough solar collector (PTSC) was estimated experimentally. The effect of structure oscillations due to wind vortex shedding on solar collector performance degradation was estimated. The performance of PTSC is evaluated by using the useful heat gain and the thermal instantaneous efficiency. Experimental work to simulate the vortex shedding excitation was done. The useful heat gain and the thermal efficiency of the parabolic trough collector were calculated from experimental measurements with and without vortex loading. The prototype of the collector was fabricated for this purpose. The effect of vortex shedding at different operation condition

In this research, damping properties for composite materials were evaluated using logarithmic decrement method to study the effect of reinforcements on the damping ratio of the epoxy matrix. Three stages of composites were prepared in this research. The first stage included preparing binary blends of epoxy (EP) and different weight percentages of polysulfide rubber (PSR) (0%, 2.5%, 5%, 7.5% and 10%). It was found that the weight percentage 5% of polysulfide was the best percentage, which gives the best mechanical properties for the blend matrix. The advantage of this blend matrix is that; it mediates between the brittle properties of epoxy and the flexible properties of a blend matrix with the highest percentage of PSR. The second stage

Roller-Compacted Concrete (RCC) is a zero-slump concrete, with no forms, no reinforcing steel, no finishing and is wet enough to support compaction by vibratory rollers. Because the effectiveness of curing on properties and durability, the primary scope of this research is to study the effect of various curing methods (air curing, emulsified asphalt(flan coat) curing, 7 days water curing and permanent water curing) and different porcelanite (local material used as an Internal Curing agent) replacement percentages (volumetric replacement) of fine aggregate on some properties of RCC and to explore the possibility of introducing more practical RCC for road pavement with minimum requirement of curing. Cubes specimens were sawed from the slab

  The current study presents the simulative study and evaluation of MANET mobility models over UDP traffic pattern to determine the effects of this traffic pattern on mobility models in MANET which is implemented in NS-2.35 according to various performance metri (Throughput, AED (Average End-2-end Delay), drop packets, NRL (Normalize Routing Load) and PDF (Packet Delivery Fraction)) with various parameters such as different velocities, different environment areas, different number of nodes,  different traffic rates, different traffic sources, different pause times and different simulation times .  A routing protocol.…was exploited AODV(Adhoc On demand Distance Vector) and RWP (Random Waypoint), GMM (Gauss Markov Model), RPGM (Refere

The aim of the research is try to answer the questions about the importance of the participation of scientists in the scientific researcher Google Scholar for the professor and the university, and the extent of participation of Iraqi universities, and the rank of the University of Qadisiyah, and the number of participants and teachers of this engine, The research came out with several conclusions, the most important of which is that (21%) of university professors are involved in the google engine and most of the participants did not carry their full research through the site