In vitro antitumor activity of Gracilaria corticata (a red alga) against Jurkat and molt-4 human cancer cell lines

Gracilaria corticata is a red alga which can be collected from many sea coasts around the world such as China, India, Persian Gulf, etc. The Persian Gulf is a unique marine habitat infested with diverse seaweeds. The aim of the present study is to explore anticancer potential of the crude extracts from G. corticata which was collected from the Bushehr coast (South west of Iran). Here, different concentration of the aqueous extract from G. corticata was tested for probable antitumoral activity on Jurkat and molt- 4 human lymphoblastic leukemic cell lines. The cells were treated by different concentration of algal extract and the number of viable cells was determined by trypan blue. Also, cytotoxicity of the extract was evaluated by methyl thiazolyl tetrazolium (MTT) assay. The results showed that 9.336 and 9.726 μg/μl of algal extract were the most effective concentrations against Jurkat and molt-4 cells, respectively. The water crude extract of red alga G. corticata had significant anticancer activity and it might be a good candidate for further investigations in order to develop a natural compound as an anticancer agent which can be used for the production of potential anticancer drug and novel pharmaceutical leads.Key words: Gracilaria corticata, anticancer, Jurkat, molt-4

Delay in Diagnosis of Two Siblings with Severe Ocular Problems and Autoimmune Polyglandular Syndrome

Autoimmune polyendocrine syndrome type 1 (APS1) is a scarce polyendocrinopathy with autosomal recessive inheritance results from defects in the human autoimmune regulatory (AIRE) gene. In addition to three major manifestations of APS1 including mucocutaneous candidiasis, hypoparathyroidism, and Addison's disease, ophthalmic problems such as keratoconjunctivitis, dry eye, iridocyclitis, and cataract can be seen in these patients. In this article, we introduced two siblings presented with nail dystrophia, severe photophobia, and keratitis since early childhood which genetic examination revealed single nucleotide T>C translocation in their 2nd exon and heterozygous deletion mutation in their 12th exon

Monogenic Primary Immunodeficiency Disorder Associated with Common Variable Immunodeficiency and Autoimmunity

Background: Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency disorder mainly characterized by recurrent bacterial infections besides other immunological defects including loss of or dysfunction of B cells and decreased immunoglobulin levels. In this study, our aim is to evaluate clinical, immunological, and molecular data of patients with a primary clinical diagnosis of CVID and autoimmune phenotype with a confirmed genetic diagnosis. Methods: Among 297 patients with CVID, who were registered in the Iranian Primary Immunodeficiency Registry at Children's Medical Center Hospital in Iran, 83 patients have been genetically examined and 27 patients with autoimmunity and confirmed genetic mutations were selected for analysis. Whole-exome sequencing and confirmatory Sanger sequencing methods were used for the study population. A questionnaire was retrospectively filled for all patients to evaluate demographic, laboratory, clinical, and genetic data. Results: In the 27 studied patients, 11 different genetic defects were identified, and the most common mutated gene was LRBA, reported in 17 (63.0) patients. Two patients (7.7) showed autoimmune complications as the first presentation of immunodeficiency. Eleven patients (40.7) developed one type of autoimmunity, and 16 patients (59.3) progressed to poly-autoimmunity. Most of the patients with mono-autoimmunity (n = 9, 90.0) primarily developed infectious complications, while in patients with poly-autoimmunity, the most common first presentation was enteropathy (n = 6, 37.6). In 13 patients (61.9), the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency. The most frequent autoimmune manifestations were hematologic (40.7), gastrointestinal (48.1), rheumatologic (25.9), and dermatologic (22.2) disorders. Patients with poly-autoimmunity had lower regulatory T cells than patients with mono-autoimmunity. Conclusion: In our cohort, the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency in most patients. This association highlights the fact that patients referring with autoimmune manifestations should be evaluated for humoral immunity. © 2020 Georg Thieme Verlag. All rights reserved

A rare case of hyper IgE syndrome with vocal cords involvement

Hyperimmunoglobulin E syndrome (HIGE) is considered as a phagocytic or a newly classified complex and heterogeneous primary immunodeficiency disease with symptoms such as increased levels of immunoglobulin E, eczema, and, recurrent lung and skin infections. In this paper, we have presented a rare case of this syndrome. A 9-year-old Iranian girl presented with a history of pruritic maculopapular rash who was eventually diagnosed as a case of HIGE. In her recent admission, she had dysphonia, stridor and huge cauliflower cutaneous lesions on her neck, finger and vocal cords, which did not respond to intravenous antibiotics, and ultimately required surgical removal

Crystal structure of native and a mutant of Lampyris turkestanicus luciferase implicate in bioluminescence color shift.

Firefly bioluminescence reaction in the presence of Mg(2+), ATP and molecular oxygen is carried out by luciferase. The luciferase structure alterations or modifications of assay conditions determine the bioluminescence color of firefly luciferase. Among different beetle luciferases, Phrixothrix hirtus railroad worm emits either yellow or red bioluminescence color. Sequence alignment analysis shows that the red-emitter luciferase from Phrixothrix hirtus has an additional arginine residue at 353 that is absent in other firefly luciferases. It was reported that insertion of Arg in an important flexible loop350-359 showed changes in bioluminescence color from green to red and the optimum temperature activity was also increased. To explain the color tuning mechanism of firefly luciferase, the structure of native and a mutant (E354R/356R/H431Y) of Lampyris turkestanicus luciferase is determined at 2.7{Angstrom} and 2.2{Angstrom} resolutions, respectively. The comparison of structure of both types of Lampyris turkestanicus luciferases reveals that the conformation of this flexible loop is significantly changed by addition of two Arg in this region. Moreover, its surface accessibility is affected considerably and some ionic bonds are made by addition of two positive charge residues. Furthermore, we noticed that the hydrogen bonding pattern of His431 with the flexible loop is changed by replacing this residue with Tyr at this position. Juxtaposition of a flexible loop (residues 351-359) in firefly luciferase and corresponding ionic and hydrogen bonds are essential for color emission

The Effect(s) of Vitamin A on Persistent Wheezing after Bronchiolitis in Infants Aged 1-12 Months

Background & aim: Viral bronchiolitis is one of the most common causeS of lower respiratory tract infection and almost responsible for 1-3% of hospitalization among children under one year of age. Post bronchiolitis wheezing is the main risk factor for childhood asthma, and its control can decrease incidence of the disease in future. The purpose of this study was to investigate the effect of vitamin A on persistent wheezing after bronchiolitis. Methods: The present double blind clinical trial was conducted on 84 patients aged 1 to 12 months of age who were admitted in Imam Sajad Hospital of Yasuj from October 2012 to 2013. After viral bronchiolitis diagnosis of patients with history and physical exam, they were divided into two similar groups of control and case randomly. Control group received cold and wet nebulized oxygen and bronchodilator and case group received the same protocol together with 5000 IU/kg Vitamin A intramascularlly. Both groups were examined by the same physician before, one and two weeks after treatment and quality and severity of wheezing was recorded. Data were analyzed by statistical student T – test and chi square test. Results: Intensity and quality of wheezing was similar in both groups before the intervention(85/0 = p=0.858) whereas a significant reduction was observed in the severity of wheezing in the group receiving vitamin A compared with the control group (05/0> p<0.05). Conclusion: A significant decrease was observed in persistent wheezing after intramascular administration of a dose of vitamin A in patients requiring hospitalization due to viral bronchiolitis. Administrating one dose vitamin A intramuscularly in patients who need hospitalization due to bronchiolitis, may decrease post bronchiolitis and persistent wheezing