Beta-thalassemia is the most common hereditary disease in Iran. More than two million carriers of beta-thalassemia live in Iran. Since the Iranian population is a mixture of different ethnic groups, it is necessary to determine the frequency and distribution of mutations in the different parts of the country. For this purpose, we divided Iran in to eight different regions according to the geographic and ethnic distribution of the population. Over a 10-year period 1,217 beta-thalassemia chromosomes of 164 affected patients and 889 unrelated carriers were studied using the amplification refractory mutation system-polymerase chain reaction technique. We detected 81% beta-thalassemia mutations in the studied chromosomes. IVS-II-I (G --> A) was the predominant mutation found in our study (34%). Its relative frequency in the north was much higher than other regions, and it lessened toward the south, where the IVS-I-5 (G --> C) mutation was more common. IVS-I-5 (G --> C) (7.55%), codons 8/9 (+ G) (4.76%), and IVS-I-110 (G --> A) (4.76%) were the other most common mutations. The results presented here can be used as a basis of prenatal diagnosis of beta-thalassemia in different regions of Iran.
Mesoporous silica nanocarriers as accommodate drug molecule capsules were synthesized and capped by chitosan natural polymer. This nanocarrier acts as a pH-responsive shield to increase the solubility and improvement of anticancer properties of curcumin against U87MG glioblastoma cancer cell line. The encapsulation efficiency and drug-loading content were measured 88.1 ± 4.76% and 8.81 ± 0.47%, respectively. The curcumin release from the CS-MCM-41 was slow and sustained at low pH (42.72 ± 2.29%) compared to the environment pH (19.54 ± 1.36%) in 96 h. The MTT evaluations showed that IC after 72 h treatment with free curcumin and curcumin-loaded CS-MCM-41 were 15.20 and 5.21 μg/mL (p <0.05). respectively.
ObjectiveTo investigate whether daily diet provides adequate selenium intake in healthy men and women living in Tehran, Iran.MethodSerum level of selenium was determined in 184 healthy individuals of both genders. The samples were divided into two age groups, adults and children, for analysis. The serum level of selenium was determined using hydride generation and flame atomic absorption spectroscopy.ResultsThe mean and standard deviation of serum selenium levels in children (1–16 years) was 84.3 ± 11 μg/l and there was no significant difference between genders in this group. In adults (older than 16 years) the mean serum selenium level was 100.6 ± 13 SD μg/l; among women the mean was 93.9 ± 14 SD μg/l and among men it was 102.2 ± 12 SD μg/l. The mean selenium level in men was higher than in women and data analysis showed a significant difference between them (p < 0.005). There was also a positive correlation between higher selenium serum concentration and age in men (P < 0.001). Daily intake of selenium in men and women was calculated to be 67 μg and 62.1 μg respectively.ConclusionOur results show that the serum concentration of selenium in an Iranian population is similar to other nationalities in the Middle East, particularly Saudi Arabia.
Background: X-linked agammaglobulinemia (XLA) is a hereditary immunodeficiency characterized by an early onset of recurrent bacterial infections, a profound deficiency of all immunoglobulin isotypes and a markedly reduced number of peripheral B lymphocytes. Eighty-five percent of the patients with this phenotype have mutations in Bruton’s tyrosine kinase (BTK) gene. Methods: To provide an informative outlook of clinical and immunological manifestations of XLA in Iran, 37 Iranian male patients with an age range of 1–34 years, followed over a period of 25 years, were studied. Twenty-four of the 37 patients were screened for BTK gene mutation using PCR-SSCP followed by direct sequencing. BTK protein expression assay was done by flow cytometry in 9 families. Results: All patients first presented with infectious diseases, the most common of which were respiratory tract infections. Eighteen different mutations were identified, 13 of which were novel: IVS1+5G>C, 1896G>A, 349delA, 1618C>T, 1783T>C, 2084A>G, 1346delT, 1351delGAG, 587A>G, IVS14–1G>A, IVS3+2T>C, 1482G>A, 1975C>A. Conclusion: The fact that we found a great number of novel mutations in a relatively limited number of patients underlines the heterogeneity of BTK mutations in the Iranian population. The large number of new mutations indicates that extended studies in this region would be rewarding.
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