Serum Chitotriosidase level as a Novel Biomarker for Therapeutic Monitoring of Nephropathic Cystinosis among the Iraqi children

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 inflammatory conditions which suggest that cystinosis should be included within the differential diagnosis of disorders associated with increased plasma chitotriosidase activity. This study is aimed to estimate serum chitotriosidase level, as a screening marker and therapeutic monitor for cystinosis disease in Iraqi children with cystinosis.Subjects and Methods: The present study is a case-control study that included samples of 30 children with nephropathic cystinosis, compared to 25 healthy control children from those attending at The Genetic Rare Diseases Center / AL-Emamain AL-Kadhimain Teaching Hospital, Baghdad-Iraq.Results: Our results reported that cystinotic children had a marked elevation of serum chitotriosidase activity, compared to age-matched healthy children, besides a significant associated with leukocyte-cystine content for cystinotic patients.CHT1 as a Novel BiomarkerConclusion: Estimation of serum chitotriosidase activity might aid in monitoring the therapeutic benefits of cysteamine therapy, as well as the prognosis of the disease when WBC cystine assessment is not available.Key Words: Cystinosis, Cysteamine, Chitotriosidase.

RESPONSE TO METHIONINE SUPPLEMENTATION OF BROILER CHICKS FED PRACTICAL CORN-GROUNDNUT MEAL DIETS IN THE TROPICS

Two floor feeding trials, involving 1000 broiler chicks fed two different practical-type diets, were conducted in order to determine the methionine and total sulphur amino acid (TSAA) requirements of broiler chicks 0-6 weeks). Two protein levels (20 and 23%) each with an energy concentration of 2800 Kcal ME (kg diet and four supplemental methionine levels (0.1, 0.2, 0.3 and 0.4%) were tested. At the end of 6 - week feeding period, a common finisher ration was fed to 10 weeks of age. Under our experimental circumstances with dietshaving 23% protein and metabolizable energy of 2800 Kcal ME/kg diet and based on assumed methionine and cystine content of feeding stuff, the quantitative requirement for methionine of broiler chicks up to six weeks of age were about 0.53% diet which is equivalent to 2.3% of the dietary protein (or TSSA of 0.85 per cent diet, that is 3.7 per cent protein). Higher supplemental methionine at this protein and energy levels resulted in deterioration in liveweight gain and slight but non-significant improvement in feed/gain ratio. The rations containing 20% protein performed poorly relative to those containing 23%. Feeding of high quality common finisher rations neutralized the initial gains due to supplemental methionine within each protein levelbut did not offset the gains due to different dietary proteins.

Study on the Structure and Properties of Biofunctional Keratin from Rabbit Hair

Keratin is widely recognized as a high-quality renewable protein resource for biomedical applications. A large amount of rabbit hair waste is produced in textile industries, because it has high medullary layer content, but poor spinnability. Therefore, it is of great significance to extract keratin from waste rabbit hair for recycling. In this research, an ultrasonic-assisted reducing agent-based extraction method was developed and applied to extract keratin from rabbit hair. The results showed that the ultrasonic treatment had a certain destructive effect on the structure of the fiber, and when combined with reducing agent, it could effectively promote the dissolution of rabbit hair, and extract keratin with high molecular weight between 31 and 94 kDa. The structure and properties of keratin were studied. Compared to the rabbit hair, the cystine content of keratin was significantly reduced, and the secondary structure changed from α-helix to β-sheet. The keratin products show excellent biocompatibility and antioxidant capacity. In addition, large keratin particles can be formed by assembly with a balance between intermolecular hydrophobic attraction as the concentration of urea in keratin solution decreased during dialysis.

Possible Participation of β-Cyanoalanine Synthase in Increasing the Amino Acid Pool of Cocklebur Seeds in Response to Ethylene during the Pre-germination Period

The amino acid content in cocklebur (Xanthium pennsylvanicum Wallr.) seeds was increased by ethylene, which stimulated their germination, regardless of whether they were non-dormant or secondarily dormant. This increase in amino acid content coincided with the increased activities of β-cyanoalanine synthase (CAS, EC 4.4.1.9) in response to ethylene. KCN and/or cysteine, the substrates of CAS, also increased the amino acid content in both non-dormant and secondarily dormant cocklebur seeds. The degrees of the increased amino acid content corresponded roughly to the germination rates of the seeds reported previously. The actual involvement of CAS in the germination process in cocklebur seeds was demonstrated by incorporation into asparagine and aspartate from 14CN which was fed to the cotyledon segments of both non-dormant and secondarily dormant cocklebur seeds. In this case, the incorporation of 14CN was augmented by ethylene, and incorporated more abundantly in the cotyledons of secondarily dormant seeds. Moreover, ethylene decreased the cysteine + cystine content in both the axial and cotyledon tissues, but increased asparagine and aspartate regardless of whether they were non-dormant or secondarily dormant. This suggests that CAS responsiveness to ethylene participates in supplying asparagine and aspartate and in increasing the amino acid pool of cocklebur seeds during the pre-germination period.