Displaying publications 81 - 100 of 156 in total

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  1. Izwan Mohd Lazim M, Safinar Ismail I, Shaari K, Abd Latip J, Ali Al-Mekhlafi N, Morita H
    Chem Biodivers, 2013 Sep;10(9):1589-96.
    PMID: 24078592 DOI: 10.1002/cbdv.201200391
    A chemical investigation of the alkaloidal fraction of Dysoxylum acutangulum leaves led to the isolation and characterization of two new chromone alkaloid analogs named chrotacumines E and F (1 and 2, resp.). Structure elucidation of 1 and 2 was achieved by spectroscopic analyses, including 2D-NMR. Both of these alkaloids exhibited modest activities as tyrosinase inhibitors with 29.2 and 25.8% inhibition at 100 μg/ml, respectively.
  2. Sulaiman S, Azis RS, Ismail I, Man HC, Yusof KFM, Abba MU, et al.
    Nanoscale Res Lett, 2021 Nov 27;16(1):168.
    PMID: 34837537 DOI: 10.1186/s11671-021-03622-y
    In this study, magnetite nano-adsorbent (MNA) was extracted from mill scale waste products, synthesized and applied to eliminate Cu2+ from an aqueous solution. Mill scale waste product was ground using conventional milling and impacted using high-energy ball milling (HEBM) for varying 3, 5, and 7 milling hours. In this regard, the prepared MNA was investigated using X-ray diffraction (XRD), high-resolution transmission electron microscope (HRTEM), field emission scanning electron microscopy-energy-dispersive X-ray spectroscopy (FESEM-EDS), UV-Vis spectroscopy, Fourier-transform infrared (FTIR), Brunauer-Emmett-Teller (BET) and zeta potential. The resultant MNA-7 h milling time displayed a crystalline structure with irregular shapes of 11.23 nm, specific surface area of 5.98 m2g-1, saturation magnetization, Ms of 8.35 emug-1, and isoelectric point charge at pH 5.4. The optimum adsorption capacity, qe of 4.42 mg.g-1 for the removal of Cu2+ ions was attained at 120 min of contact time. The experimental data were best fitted to the Temkin isotherm model. A comparison between experimental kinetic studies and the theoretical aspects showed that the pseudo-second-order matched the experimental trends with a correlation coefficient of (R2 > 0.99). Besides, regeneration efficiency of 70.87% was achieved after three cycles of reusability studies. The MNA offers a practical, efficient, low-cost approach to reutilize mill scale waste products and provide ultra-fast separation to remove Cu2+ from water.
  3. Almaleeh AA, Zakaria A, Kamarudin LM, Rahiman MHF, Ndzi DL, Ismail I
    Sensors (Basel), 2022 Jan 05;22(1).
    PMID: 35009947 DOI: 10.3390/s22010405
    The moisture content of stored rice is dependent on the surrounding and environmental factors which in turn affect the quality and economic value of the grains. Therefore, the moisture content of grains needs to be measured frequently to ensure that optimum conditions that preserve their quality are maintained. The current state of the art for moisture measurement of rice in a silo is based on grab sampling or relies on single rod sensors placed randomly into the grain. The sensors that are currently used are very localized and are, therefore, unable to provide continuous measurement of the moisture distribution in the silo. To the authors' knowledge, there is no commercially available 3D volumetric measurement system for rice moisture content in a silo. Hence, this paper presents results of work carried out using low-cost wireless devices that can be placed around the silo to measure changes in the moisture content of rice. This paper proposes a novel technique based on radio frequency tomographic imaging using low-cost wireless devices and regression-based machine learning to provide contactless non-destructive 3D volumetric moisture content distribution in stored rice grain. This proposed technique can detect multiple levels of localized moisture distributions in the silo with accuracies greater than or equal to 83.7%, depending on the size and shape of the sample under test. Unlike other approaches proposed in open literature or employed in the sector, the proposed system can be deployed to provide continuous monitoring of the moisture distribution in silos.
  4. Adli A, Wahab AA, Abdul Latiff AH, Ismail IH, Faizah MZ, Boekhren KB, et al.
    Med J Malaysia, 2022 Jan;77(1):95-97.
    PMID: 35087003
    We report a clinical and laboratory observation in a boy with X-linked agammaglobulinemia (XLA) who underwent an immunoglobulin replacement therapy (IRT) via the subcutaneous route (IGSC) seven years after his IRT via intravenous route (IGIV). He was free of invasive infections when on IGIV but not the troublesome coughs a week before the next infusion. A switch to a subcutaneous route resulted in significant improvement of symptoms with good weight gain. When on 2-weekly IGSC cycle, adjusting dose for weight resulted in an IgG trough level of > 600 mg/dl.
  5. Nasir HM, Wee SY, Aris AZ, Abdullah LC, Ismail I
    Chemosphere, 2022 Mar;291(Pt 1):132726.
    PMID: 34718023 DOI: 10.1016/j.chemosphere.2021.132726
    Persistent endocrine-disrupting compounds (EDCs) in bodies of water are a concern for human health and constitute an environmental issue, even if present in trace amounts. Conventional treatment systems do not entirely remove EDCs from discharge effluent. Due to the ultra-trace level of EDCs which affect human health and pose an environmental issue, developing new approaches and techniques to remove these micropollutants from the discharged effluent is vital. This review discusses the most common methods of eliminating EDCs through preliminary, primary, secondary and tertiary treatments. The adsorption process is favoured for EDC removal, as it is an economical and straightforward option. The NABC aspects, which are the need, approach, benefits and challenges, were analysed based on existing circumstances, highlighting biochar as a green and renewable adsorbent for the removal of organic contaminants. From the environmental point of view, the effectiveness of this method, which uses natural fibre from the kenaf plant as a porous and economical biochar material with a selected lignocellulosic biomass, provides insights into the advantages of biochar-derived adsorbents. Essentially, the improvement of the natural fibre as an adsorbent is a focus, using carbonisation, activation, and the physiochemical process to enhance the adsorption ability of the material for pollutants in bodies of water. This output will complement sustainable water management approaches presented in previous studies for combating the emerging pollutant crisis via novel green and environmentally safe options.
  6. Nadarajah K, Mat Razali N, Cheah BH, Sahruna NS, Ismail I, Tathode M, et al.
    Genome Announc, 2017 Oct 26;5(43).
    PMID: 29074665 DOI: 10.1128/genomeA.01188-17
    Sheath blight, caused by Rhizoctonia solani anastomosis group 1 subgroup 1A (AG1-1A), is one of the most devastating rice diseases worldwide. Here, we report the draft genome sequence of R. solani AG1-1A strain 1802/KB isolated from a popular Malaysian rice variety. To the best of our knowledge, this is the second reported representative genome from AG1-1A.
  7. Tan LW, Tan CS, Rahman ZA, Goh HH, Ismail I, Zainal Z
    Data Brief, 2017 Oct;14:267-271.
    PMID: 28795104 DOI: 10.1016/j.dib.2017.07.047
    The dataset presented in this article describes microarray experiment of Auxin-binding protein 57, Abp57-overexpressing transgenic rice. The gene expression profiles were generated using Affymetrix GeneChip® Rice (Cn) Gene 1.0 ST Arrays. Total RNA from seedlings tissue of transgenic rice and wildtype, which serve as control were used as starting materials for microarray experiment. Detailed experimental methods and data analysis were described here. The raw and normalized microarray data were deposited into Gene Expression Omnibus (GEO) under accession number GSE99055.
  8. Toni B, Monfared HH, Mat Isa MN, Md Isa N, Ismail I, Zainal Z
    Data Brief, 2017 Oct;14:260-266.
    PMID: 28795103 DOI: 10.1016/j.dib.2017.07.043
    Drought stress is the main abiotic factor affecting rice production. Rain-fed upland rice which is grown on unbounded fields and totally dependent on rainfall for moisture is more prone to drought stress compared to rice from other ecosystems. However, upland rice has adapted to this limited water condition, thus are more drought tolerant than rice from other ecosystems. We performed the first transcriptome sequencing of drought tolerant indica upland rice cultivar Kuku Belang to identify differentially expressed genes related to drought tolerance mechanism. Raw reads for non-treated and PEG-treated Oryza sativa subspecies indica cv. Kuku Belang were deposited in the NCBI SRA database with accession number SRP074520 (https://www.ncbi.nlm.nih.gov/sra?term=SRP074520).
  9. Samad AFA, Nazaruddin N, Murad AMA, Jani J, Zainal Z, Ismail I
    3 Biotech, 2018 Mar;8(3):136.
    PMID: 29479512 DOI: 10.1007/s13205-018-1164-8
    In current era, majority of microRNA (miRNA) are being discovered through computational approaches which are more confined towards model plants. Here, for the first time, we have described the identification and characterization of novel miRNA in a non-model plant, Persicaria minor (P. minor) using computational approach. Unannotated sequences from deep sequencing were analyzed based on previous well-established parameters. Around 24 putative novel miRNAs were identified from 6,417,780 reads of the unannotated sequence which represented 11 unique putative miRNA sequences. PsRobot target prediction tool was deployed to identify the target transcripts of putative novel miRNAs. Most of the predicted target transcripts (mRNAs) were known to be involved in plant development and stress responses. Gene ontology showed that majority of the putative novel miRNA targets involved in cellular component (69.07%), followed by molecular function (30.08%) and biological process (0.85%). Out of 11 unique putative miRNAs, 7 miRNAs were validated through semi-quantitative PCR. These novel miRNAs discoveries in P. minor may develop and update the current public miRNA database.
  10. Nazaruddin N, Samad AFA, Sajad M, Jani J, Zainal Z, Ismail I
    Genom Data, 2017 Jun;12:157-158.
    PMID: 28560166 DOI: 10.1016/j.gdata.2017.05.011
    Persicaria minor (Kesum) is an important medicinal plant with high level of secondary metabolite contents, especially, terpenoids and flavonoids. Previous studies have revealed that application of exogenous phytohormone could increase secondary metabolite contents of the plant. MicroRNAs (miRNAs) are small RNAs that play important regulatory roles in various biological processes. In order to explore the possible role of miRNA in the regulation of these phytohormones signaling pathway and uncovering their potential correlation, we, for the first time, have generated the smallRNA library of Kesum plant. The library was developed in response to methyl jasmonate (MJ) and abscisic acid (ABA) treatment by using next-generation sequencing technology. Raw reads have been deposited to SRA database with the accession numbers, SRX2655642 and SRX2655643 (MJ-treated), SRXSRX2655644 and SRX2655645 (ABA-treated) and SRX2655646and SRX2655647 (Control).
  11. Samad AFA, Nazaruddin N, Sajad M, Jani J, Murad AMA, Zainal Z, et al.
    Genom Data, 2017 Sep;13:3-4.
    PMID: 28560169 DOI: 10.1016/j.gdata.2017.05.014
    Persicaria minor (kesum) is an important medicinal plant and commonly found in southeast countries; Malaysia, Thailand, Indonesia, and Vietnam. This plant is enriched with a variety of secondary metabolites (SMs), and among these SMs, terpenoids are in high abundance. Terpenoids are comprised of many valuable biomolecules which have well-established role in agriculture and pharmaceutical industry. In P. minor, for the first time, we have generated small RNAs data sets, which can be used as tool in deciphering their roles in terpenoid biosynthesis pathways. Fungal pathogen, Fusarium oxysporum was used as elicitor to trigger SMs biosynthesis in P. minor. Raw reads and small RNA analysis data have already been deposited at GenBank under the accessions; SRX2645684 (Fusarium-treated), SRX2645685 (Fusarium-treated), SRX2645686 (mock-infected), and SRX2645687 (mock-infected).
  12. Liu C, Liu L, Huang Y, Shi R, Wu Y, Hakimah Binti Ismail I
    Int Immunopharmacol, 2023 Jan;114:109493.
    PMID: 36527879 DOI: 10.1016/j.intimp.2022.109493
    Minimal change disease (MCD) is a common type of nephrotic syndrome with high recurrence rate. This study aims to explore the impacts of interleukin (IL)-33 in MCD and to discuss its potential mechanism. In adriamycin (ADM) and puromycin aminonucleoside (PAN)-induced MCD rat model, IL-33 was used for treatment. H&E staining was applied for detecting histological changes. Critical proteins were examined by western blot. Corresponding commercial kits tested oxidative stress- and inflammation-related factors. Cell apoptosis was measured by TUNEL assay. ADM-induced podocyte injury model was establish to mimic MCD in vitro. Cell proliferation and apoptosis were detected by CCK-8 and TUNEL assays. Finally, podocyte was stimulated by innate lymphoid type-2 cells-secreted Th2 cytokines (ILC2s: IL-13 and IL-5 respectively), with or without incubation with M1 macrophage medium to further explore the immune-regulation of ILC2s behind the inflammatory environment of MCD. It was found that PAN-induced kidney jury, inflammation, oxidative stress and apoptosis were severer than ADM, and IL-33 treatment significantly alleviated the above injuries in PAN and ADM-induced MCD rat model. Moreover, IL-33 reversed the reduced viability and increased oxidative stress and apoptosis in ADM-induced podocyte injury model. Further, the capacities of IL-13 alone in inducing M1/M2 macrophage polarization, apoptosis, inflammation, kidney injury and reducing cell viability are stronger than IL-5. However, IL-13 reversed reduced cell viability and stimulated apoptosis, inflammation, kidney injury mediated by co-incubation with M1-conditioned medium. Collectively, IL-33 might protect against immunologic injury in MCD via mediating ILC2s-secreted IL-13.
  13. Razman NA, Wan Ismail WZ, Abd Razak MH, Ismail I, Jamaludin J
    Int J Environ Sci Technol (Tehran), 2023;20(4):3789-3800.
    PMID: 35729914 DOI: 10.1007/s13762-022-04192-x
    Water pollution always occurs in Malaysia due to industrial, construction, agriculture, and household activities. River pollution can disturb water supply that eventually affects business and household activities. Thus, water quality monitoring system is needed to detect contaminated water. We developed a water quality monitoring and filtration system controlled by Arduino. The proposed system was designed in Proteus software and ThingSpeak platform was used for real-time monitoring. The main objective of the study was to compare water quality of river, lake and tap water in terms of pH, temperature, turbidity, electrical conductivity and oxidation-reduction potential. If the water quality was not satisfied, the water sample would be filtered through filtration system. Water turbidity level, pH, temperature, electrical conductivity, and oxidation-reduction potential for filtered and nonfiltered water were compared and analyzed according to international and national water quality standard. Besides that, statistical analysis such as box plot and one-way analysis of variance test was applied to validate data from the system. The real-time water quality monitoring system was implemented through data storage, data transfer, and data processing. The system was connected to wireless fidelity whereas the output data was sent to the user and monitored by ThingSpeak. The system can be further upgraded and scaled up to be applied in the main tank at our home or factory. The outcome of this research can be used as a reference for further study on lake and river pollution monitoring system.

    SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s13762-022-04192-x.

  14. Abdullah NH, Mustaffa MS, Hamidon MN, Shafie FN, Ismail I, Ibrahim IR
    Sci Rep, 2021 Oct 04;11(1):19642.
    PMID: 34608254 DOI: 10.1038/s41598-021-99236-6
    A new approach through heat treatment has been attempted by establishing defects by the process of quenching towards electrical and magnetic properties in the nickel zinc ferrite (Ni0.5Zn0.5Fe2O4) sample. The measured property values in permeability and hysteresis characteristic gave their recovery behaviour in which the values, after quenching were recovered after undergoing the annealing. Interestingly, a different trend observed in the permittivity value whereas the value was increased after quenching and subsequently recovered after annealing. The mechanisms which produced the changes is believed to be involved by defects in the form of vacancies, interstitials, microcracks and dislocations created during quenching which gave rise to changes in the values of the complex permeability and permittivity components and hysteresis behaviour.
  15. Saputra DEE, Maulida Rahma V, Eliyana A, Pratama AS, Anggraini RD, Kamil NLM, et al.
    PLoS One, 2023;18(6):e0285293.
    PMID: 37352227 DOI: 10.1371/journal.pone.0285293
    This study aims to analyze the influence of the system quality and information quality from the Correctional Database System (CDS) on the job performance of correctional Technical Support Officers (TSO) in Sumatra, either directly or through the mediating role of perceived ease of use and perceived usefulness. This study tested 118 correctional officers selected by the purposive sampling technique. A quantitative approach is used with Partial Least Square-Structural Equation Modeling analysis technique. It was found that the direct influence of the system and information quality on job performance is insignificant. Nonetheless, it shows that perceived ease of use and perceived usefulness fully mediate system quality and information quality on job performance. The findings demonstrate that perceived ease of use and perceived usefulness are important factors that motivate individual performance when supported by high-quality information systems. This study provides an overview of the use of a mandatory system for public organizations and the effects on user performance. It can be used as a basis for correctional institutions to strengthen and maintain a positive perception of CDS among their officers, such as through mentoring programs.
  16. Kho SS, Lim KC, Muhammad NA, Nasaruddin MZ, Ismail I, Daut UM, et al.
    Med J Malaysia, 2023 Mar;78(2):131-138.
    PMID: 36988520
    INTRODUCTION: COVID-19 patients frequently demonstrate radiological organising pneumonia (OP) pattern. The longterm outcome and treatment options for this group of patients remain uncertain. We aim to describe the clinical and radiological outcomes of patients with COVID-19-related OP and identify possible clinical factors associated with inferior radiological outcome.

    MATERIALS AND METHODS: Post-COVID-19 clinic attendees, consisting of post-COVID-19 patients discharged from major hospitals in the state of Selangor during the third pandemic wave of COVID-19 in Malaysia, were enrolled in this retrospective study for 6 months. Physician-scored Modified Medical Research Council (mMRC), patient self-reported quality of life (EQ-VAS) score and follow-up CT scan were evaluated.

    RESULTS: Our cohort comprised 131 patients, with a median age of 52 (IQR 39-60) years and median BMI of 29.40 (IQR 25.59-34.72). Majority (72.5%) had co-morbidities, and 97.7% had severe disease requiring supplementary oxygen support during the acute COVID-19 episode. 56.5% required intensive care; among which one-third were invasively ventilated. Median equivalent dose of methylprednisolone prescribed was 2.60 (IQR 1.29-5.18) mg/kg during admission, while the median prednisolone dose upon discharge was 0.64 (IQR 0.51-0.78) mg/kg. It was tapered over a median of 8.0 (IQR 5.8-9.0) weeks. Upon follow-up at 11 (IQR 8-15) weeks, one-third of patients remained symptomatic, with cough, fatigue and dyspnoea being the most reported symptoms. mMRC and EQ-VAS scores improved significantly (p<0.001) during follow-up. Repeat CT scans were done in 59.5% of patients, with 94.8% of them demonstrating improvement. In fact, 51.7% had complete radiological resolution. Intensive care admission and mechanical ventilation are among the factors which were associated with poorer radiological outcomes, p<0.05.

    CONCLUSION: Approximately one-third of patients with SARSCoV- 2-related OP remained symptomatic at 3 months of follow-up. Majority demonstrated favourable radiological outcomes at 5-month reassessment, except those who required intensive care unit admission and mechanical ventilation.

  17. Batumalaie K, Zaman Safi S, Mohd Yusof K, Shah Ismail I, Devi Sekaran S, Qvist R
    Int J Endocrinol, 2013;2013:367312.
    PMID: 24324490 DOI: 10.1155/2013/367312
    Background. Oxidative stress induced by reactive oxygen and nitrogen species is critically involved in the impairment of β -cell function during the development of diabetes. Methods. HIT-T15 cells were cultured in 5% CO2 and then preincubated with Gelam honey extracts (20, 40, 60, and 80 µg/mL) as well as quercetin (20, 40, 60, and 80 µM), prior to stimulation by 20 and 50 mM of glucose. Cell lysate was collected to determine the effect of honey extracts and quercetin on the stress activated NF- κ B, MAPK pathways, and the Akt (ser473) activated insulin signaling pathway. Results. HIT-T15 cells cultured under hyperglycemic conditions demonstrated insulin resistance with a significant increase in the levels of MAPK, NF- κ B, and IRS-1 serine phosphorylation (ser307); however, Akt expression and insulin contents are significantly decreased. Pretreatment with quercetin and Gelam honey extract improved insulin resistance and insulin content by reducing the expression of MAPK, NF- κ B, and IRS-1 serine phosphorylation (ser307) and increasing the expression of Akt significantly. Conclusion. Gelam honey-induced differential expression of MAPK, NF- κ B, IRS-1 (ser307), and Akt in HIT-T15 cells shows that Gelam honey exerts protective effects against diabetes- and hyperglycemia-induced oxidative stress by improving insulin content and insulin resistance.
  18. Liew SK, Lee JA, Tamam F, Ismail II, Mohamed-Saaid F, Chye PC
    Malays Orthop J, 2023 Mar;17(1):70-78.
    PMID: 37064627 DOI: 10.5704/MOJ.2303.009
    INTRODUCTION: The awareness of under-representation of female surgeons in orthopaedics has been increasing in this decade. We aim to investigate the reasons why female surgeons chose orthopaedic, the barriers that possibly hinder female surgeons into orthopaedics and analyse the obstacles that they encountered in their career in Malaysian context.

    MATERIALS AND METHODS: A total of 101 registered female orthopaedic surgeons registered with the Malaysian Medical Council, during the period 1980 to 2020, were contacted for a cross-sectional survey, consisting of thirty-four questions on their experience in the orthopaedic career. Eighty-two responses were received (81.2%). Questions in this survey consisted of four sections: (1) demographic details, (2) current clinical practice environment, (3) orthopaedics training experience, and (4) career experience.

    RESULTS: A total of 49% of respondents had subspeciality training, highest in paediatric orthopaedic (30%). Enjoyment of manual tasks (64.6%) and professional satisfaction (64.6%) were the top reasons for choosing orthopaedic as a career. Primary barriers to orthopaedic were physical strength required (56.0%) and public gender bias (52.4%). Twenty-eight percent reported gender discrimination in career opportunities while 60% reported similar in daily work. Thirty-three percent reported verbal and 11% physical sexual harassment in their career. Forty-four percent of respondents reported benefits as female orthopaedic surgeon in their work.

    CONCLUSION: The reasons for Malaysian female orthopaedic surgeons to choose orthopaedic as their career and the barriers perceived to hinder other females from choosing orthopaedics were similar to reports worldwide with no exception to gender discrimination and sexual harassment. The support given by male colleagues to married female surgeons marked a unique phenomenon in Malaysian culture.

  19. Abraham RE, Min GL, Pauzi ALBM, Salim NHA, Ismail I
    Turk J Emerg Med, 2023;23(3):191-194.
    PMID: 37529792 DOI: 10.4103/2452-2473.367398
    Respiratory myoclonus, also known as belly dancer's dyskinesia (BDD), is a rare manifestation of movement disorder characterized by repetitive choreiform involuntary movements involving the anterior abdominal muscles, the diaphragm, and other respiratory muscles. Currently, there is no definite pathophysiology that clearly explains this condition. A 25-year-old male with a known case of BDD presented with an exacerbation of involuntary and continuous writhing movements of the abdominal wall muscles associated with abdominal pain and shortness of breath over the past 2 days. Subsequently, he was intubated due to worsening respiratory distress a few days after his admission. He was then put on ultrasound-guided botulinum toxin A injections of 25 units over the left hemidiaphragm regularly. His symptoms markedly improved since then as the attacks had reduced to 5-6 monthly intervals. Administration of ultrasound-guided botulinum toxin A injections may help to control the exacerbation of BDD and might be an option for cases refractory to medical treatment and phrenic nerve ablation.
  20. Saad HKM, Taib WRW, Ismail I, Johan MF, Al-Wajeeh AS, Al-Jamal HAN
    Exp Ther Med, 2021 Dec;22(6):1402.
    PMID: 34675995 DOI: 10.3892/etm.2021.10838
    Iron homeostasis is regulated by hepcidin (HEPC) that controls the dietary iron absorption and iron recycling. HEPC deficiency contributes to iron overload in β-thalassemia patients. The present study aimed to investigate the correlation between HEPC concentration and serum iron status among hemoglobin E (HbE)/β-thalassemia patients and their parents (HbE trait and β-thalassemia trait) compared with healthy controls. This study is a comparative cross-sectional study in which iron profile and HEPC level were examined in 65 HbE/β-thalassemia patients (pretransfusion) and 65 parents at the Hospital Sultanah Nur Zahirah and in 130 students as healthy controls from Univesiti Sultan Zainal Abidin, Terengganu, Malaysia. Furthermore, six samples from each group (HbE/β-thalassemia patients, parents and healthy controls) were randomly selected for gene expression analysis of HEPC and ferroportin1 (FPN1) using reverse transcription quantitative PCR. The results demonstrated that serum HEPC level were significantly decreased in HbE/β-thalassemia patients and their parents (P<0.001) compared with healthy controls. In addition, the gene expression analysis showed a dramatically downregulated HEPC in HbE/β-thalassemia patients and their parents (P=0.001) compared with healthy controls. However, there was a marked upregulation of FPN1 in HbE/β-thalassemia patients and their parents (P=0.001) compared with healthy controls. Iron profiling results revealed a significantly increased serum ferritin in HbE/β-thalassemia patients and their parents compared with healthy controls (P<0.001). In summary, the present study demonstrated that HEPC expression level and serum level were significantly decreased in HbE/β-thalassemia patients and their parents, which was combined with a marked increased FPN1 expression level and serum ferritin level compared with healthy volunteers. These findings supported the hypothesis that downregulated HEPC could lose its function as a negative regulator of FPN1, resulting in iron overload in HbE/β-thalassemia patients. Subsequently, assessing HEPC and FPN1 gene expression may be a useful tool to determine the risk of iron toxicity in patients with HbE/β-thalassemia and their parents, and could therefore be considered as a therapeutic target in the management of iron burden in these patients.
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