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Combined immunodeficiency

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https://www.readbyqxmd.com/read/29783100/cd133-cells-derived-from-skeletal-muscles-of-duchenne-muscular-dystrophy-patients-have-a-compromised-myogenic-and-muscle-regenerative-capability
#1
Jinhong Meng, Francesco Muntoni, Jennifer Morgan
Cell-mediated gene therapy is a possible means to treat muscular dystrophies like Duchenne muscular dystrophy. Autologous patient stem cells can be genetically-corrected and transplanted back into the patient, without causing immunorejection problems. Regenerated muscle fibres derived from these cells will express the missing dystrophin protein, thus improving muscle function. CD133+ cells derived from normal human skeletal muscle contribute to regenerated muscle fibres and form muscle stem cells after their intra-muscular transplantation into an immunodeficient mouse model...
May 12, 2018: Stem Cell Research
https://www.readbyqxmd.com/read/29780393/immunomodulatory-drugs-exert-anti-leukemia-effects-in-acute-myeloid-leukemia-by-direct-and-immunostimulatory-activities
#2
Aude Le Roy, Thomas Prébet, Rémy Castellano, Armelle Goubard, Florence Riccardi, Cyril Fauriat, Samuel Granjeaud, Audrey Benyamine, Céline Castanier, Florence Orlanducci, Amira Ben Amara, Frédéric Pont, Jean-Jacques Fournié, Yves Collette, Jean-Louis Mege, Norbert Vey, Daniel Olive
Immunomodulatory drugs (IMiDs) are anticancer drugs with immunomodulatory, anti-angiogenesis, anti-proliferative, and pro-apoptotic properties. IMiDs are currently used for the treatment of multiple myeloma, myelodysplastic syndrome, and B-cell lymphoma; however, little is known about efficacy in acute myeloid leukemia (AML). We proposed in this study to investigate the relevance of IMiDs therapy for AML treatment. We evaluated the effect of IMiDs on primary AML blasts ( n  = 24), and the impact in natural killer (NK) cell-mediated immunosurveillance of AML...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29779669/synthesis-and-profiling-of-benzylmorpholine-1-2-4-5-tetraoxane-analogue-n205-towards-tetraoxane-scaffolds-with-potential-for-single-dose-cure-of-malaria
#3
Paul M O' Neill, Paul A Stocks, Sunil Sabbani, Natalie L Roberts, Richard K Amewu, Emma R Shore, Ghaith Aljayyoussi, Iñigo Angulo-Barturén, María Belén, Jiménez-Díaz, Santiago Ferrer Bazaga, María Santos Martínez, Brice Campo, Raman Sharma, Susan A Charman, Eileen Ryan, Gong Chen, David M Shackleford, Jill Davies, Gemma L Nixon, Giancarlo A Biagini, Stephen A Ward
A series of aryl carboxamide and benzylamino dispiro 1,2,4,5-tetraoxane analogues have been designed and synthesized in a short synthetic sequence from readily available starting materials. From this series of endoperoxides, molecules with in vitro IC50s versus Plasmodium falciparum (3D7) as low as 0.84 nM were identified. Based on an assessment of blood stability and in vitro microsomal stability, N205 (10a) was selected for rodent pharmacokinetic and in vivo antimalarial efficacy studies in the mouse Plasmodium berghei and Plasmodium falciparum Pf3D70087/N9 severe combined immunodeficiency (SCID) mouse models...
May 17, 2018: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/29778502/hematopoietic-stem-cell-transplantation-for-progressive-combined-immunodeficiency-and-lymphoproliferation-in-activated-pi3k-delta-syndrome-type-1
#4
Tsubasa Okano, Kohsuke Imai, Yuki Tsujita, Noriko Mitsuiki, Kenichi Yoshida, Chikako Kamae, Kenichi Honma, Kanako Mitsui-Sekinaka, Yujin Sekinaka, Tamaki Kato, Katsuyuki Hanabusa, Eri Endo, Takehiro Takashima, Haruka Hiroki, Tzu-Wen Yeh, Keisuke Tanaka, Masakazu Nagahori, Ikuya Tsuge, Yuki Bando, Fuminori Iwasaki, Yoshiaki Shikama, Masami Inoue, Tomiko Kimoto, Naohiko Moriguchi, Yuki Yuza, Takashi Kaneko, Kyoko Suzuki, Tomoyo Matsubara, Yoshihiro Maruo, Tomoaki Kunitsu, Tomoko Waragai, Hideki Sano, Yuko Hashimoto, Kazuhiro Tasaki, Osamu Suzuki, Toshihiko Shirakawa, Motohiro Kato, Toru Uchiyama, Masataka Ishimura, Tetsuzo Tauchi, Hiroshi Yagasaki, Shiann-Tarng Jou, Hsin-Hui Yu, Hirokazu Kanegane, Sven Kracker, Anne Durandy, Daiei Kojima, Hideki Muramatsu, Taizo Wada, Yuzaburo Inoue, Hidetoshi Takada, Seiji Kojima, Seishi Ogawa, Osamu Ohara, Shigeaki Nonoyama, Tomohiro Morio
BACKGROUND: Activated phosphatidylinositol-3-OH kinase-delta (PI3Kδ) syndrome type 1 (APDS1) is a recently described primary immunodeficiency syndrome characterized by recurrent respiratory infections, lymphoid hyperplasia, and herpesviridae infections due to germline gain-of-function mutations of PIK3CD. Hematopoietic stem cell transplantation (HSCT) may be considered to ameliorate progressive immunodeficiency and associated malignancy, but appropriate indications, method, and outcomes of HSCT for APDS1 remain undefined...
May 17, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29777923/diagnosis-and-management-of-pulmonary-infection-due-to-rhodococcus-equi
#5
REVIEW
Weijie V Lin, Robert L Kruse, Katharine Yang, Daniel M Musher
BACKGROUND: Rhodococcus equi is a recognized cause of disease in humans, especially in patients who are immunocompromised. Because diphtheroids are regarded as part of normal respiratory flora, the importance of R. equi as a pulmonary pathogen may not be fully appreciated and its prevalence underestimated. Most treatment recommendations for R. equi infection were established before antiretroviral drugs became available for HIV/AIDS therapy, and therapeutic strategies may need to be updated...
May 16, 2018: Clinical Microbiology and Infection
https://www.readbyqxmd.com/read/29770060/setting-the-stage-for-expanding-hiv-pre-exposure-prophylaxis-use-in-canada
#6
EDITORIAL
M Hull, Dhs Tan
Human immunodeficiency virus (HIV) infection continues to disproportionately affect vulnerable populations in Canada; particularly men who have sex with men (MSM). Novel HIV prevention strategies have recently expanded from the use of non-occupational post-exposure prophylaxis (nPEP) after high risk exposures to the use of pre-exposure prophylaxis (PrEP) in which individuals reduce risk of HIV infection through use of combination antiretrovirals taken prior to risk exposure. With approval of tenofovir/emtricitabine (TDF/FTC) for use as PrEP only in early 2016, and with limited public funding to date, uptake in Canada is in its preliminary stages...
December 7, 2017: Canada Communicable Disease Report, Relevé des Maladies Transmissibles Au Canada
https://www.readbyqxmd.com/read/29770057/aids-in-canada-surveillance-report-2016
#7
L Jonah, A C Bourgeois, M Edmunds, A Awan, O Varsaneux, W Siu
Background: Although there continues to be a global epidemic of people living with human immunodeficiency virus (HIV) there has been a decrease in the number of people dying of acquired immunodeficiency syndrome (AIDS), largely due to successful treatment with antiretroviral therapy. Objective: To provide a descriptive overview of the reported cases of AIDS in Canada by identifying trends by geographic location, sex, age group and mortality. While the descriptive analysis focuses on the year 2016, results are presented for reported cases from the beginning of national AIDS surveillance in 1979...
December 7, 2017: Canada Communicable Disease Report, Relevé des Maladies Transmissibles Au Canada
https://www.readbyqxmd.com/read/29768476/treatment-with-specific-soluble-factors-promotes-the-functional-maturation-of-transcription-factor-mediated-pancreatic-transdifferentiated-cells
#8
Hiroaki Motoyama, Akira Kobayashi, Takahide Yokoyama, Akira Shimizu, Hiroshi Sakai, Tsuyoshi Notake, Kentaro Fukushima, Shin-Ichi Miyagawa
Pancreatic lineage-specific transcription factors (TFs) display instructive roles in converting adult cells to endocrine pancreatic cells through a process known as transdifferentiation. However, little is known about potential factors capable of accelerating transdifferentiation following transduction to achieve the functional maturation of transdifferentiated cells. In this study, we demonstrated, using adult liver-derived progenitor cells, that soluble factors utilized in pancreatic differentiation protocols of pluripotent stem cells promote functional maturation of TFs-mediated transdifferentiated cells...
2018: PloS One
https://www.readbyqxmd.com/read/29767965/bifunctional-chimera-that-coordinately-targets-human-immunodeficiency-virus-1-hiv-1-envelope-gp120-and-host-cell-ccr5-co-receptor-at-the-virus-cell-interface
#9
Adel A Rashad, Li-Rui Song, Andrew P Holmes, Kriti Acharya, Shiyu Zhang, Zhi-Long Wang, Ebony Gary, Xin Xie, Vanessa Pirrone, Michele A Kutzler, Ya-Qiu Long, Irwin Chaiken
To address the urgent need for new agents to reduce the global occurrence and spread of AIDS, we investigated the underlying hypothesis that antagonists of HIV-1 Envelope (Env) gp120 and host cell co-receptor (CoR) proteins can be covalently joined into bifunctional synergistic combinations that will improve antiviral. A synthetic protocol was established to covalently combine a CCR5 small molecule antagonist and a gp120 peptide triazole antagonist to form the bifunctional chimera. Importantly, the chimeric inhibitor preserved the specific targeting properties of the two separate chimera components, and at the same time exhibited low to sub-nanomolar potencies, in inhibiting cell infection by different pseudoviruses, that were substantially greater than those of a non-covalent mixture of individual components...
May 16, 2018: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29761856/autoimmune-thyroiditis-following-hla-matched-sibling-hematopoietic-stem-cell-transplantation-for-wiskott-aldrich-syndrome
#10
Tristan Knight, Katherine Kotz, Süreyya Savaşan
WAS is a fatal X-linked combined immunodeficiency syndrome, the only cures for which are HSCT or gene therapy. AID occur in up to 72% of patients with WAS who do not receive HSCT, likely arising secondary to impaired multilineage immune autoregulatory function; AITD is not typically seen. In this article, we describe the case of a male patient who underwent HLA-matched HSCT for WAS at the age of 5 months, with his sister (a WAS carrier) acting as his donor and subsequently developed AITD 12 months post-transplant, with marked elevation of antithyroid peroxidase antibody titer...
May 15, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29757593/severe-combined-immunodeficiency-a-case-series-and-review-from-a-tertiary-pediatric-hospital
#11
Shahrzad Fallah, Mehrnaz Mesdaghi, Mahboubeh Mansouri, Delara Babaei, Abdollah Karimi, Seyed Alireza Fahimzad, Shahnaz Armin, Sedigheh Rafiei Tabatabaei, Roxana Azma, Ghamartaj Khanbabaee, Bahram Bashardoost, Mehrdad Amirmoeini, Saeed Sadr, Rozita Jalilianhasanpour, Roxana Ghanaei, Nima Rezaei, Zahra Chavoshzadeh
Severe combined immunodeficiency syndrome (SCID) is a life-threatening condition leading to early infant death as a result of severe infection, due to impaired cellular and humoral immune systems. Various forms of SCID are classified based on the presence or absence of T cells, B cells and natural killer cells. Patients usually present with recurrent infections and failure to thrive. Definitive treatment is hematopoietic stem cell transplantation. To achieve the best outcome, it should be performed prior to the development of severe infection...
April 2018: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29756539/skin-regeneration-with-a-scaffold-of-predefined-shape-and-bioactive-peptide-hydrogels
#12
Heejung Im, Su Hee Kim, Soo Hyun Kim, Youngmee Jung
We developed a highly elastic customized scaffold for soft-tissue regeneration and combined them with bioactive hydrogels with stem-cell-inducing ability. This was done to mimic mechanical properties of native soft tissues and improve the viability of transplanted cells as well as efficiency of tissue regeneration. The proposed study was aimed at evaluating various characteristics of scaffolds and investigating their tissue regenerating ability. Finger shaped porous scaffolds were successfully fabricated by an indirect 3D printing of poly (L-lactide-co-ε-caprolactone) (PLCL) which provides a high elasticity for soft tissue engineering...
May 14, 2018: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/29753658/feasibility-and-biological-rationale-of-repurposing-sunitinib-and-erlotinib-for-dengue-treatment
#13
Szuyuan Pu, Fei Xiao, Stanford Schor, Elena Bekerman, Fabio Zanini, Rina Barouch-Bentov, Claude M Nagamine, Shirit Einav
There is an urgent need for strategies to combat dengue virus (DENV) infection; a major global threat. We reported that the cellular kinases AAK1 and GAK regulate intracellular trafficking of multiple viruses and that sunitinib and erlotinib, approved anticancer drugs with potent activity against these kinases, protect DENV-infected mice from mortality. Nevertheless, further characterization of the therapeutic potential and underlying mechanism of this approach is required prior to clinical evaluation. Here, we demonstrate that sunitinib/erlotinib combination achieves sustained suppression of systemic infection at approved dose in DENV-infected IFN-α/β and IFN-γ receptor-deficient mice...
May 10, 2018: Antiviral Research
https://www.readbyqxmd.com/read/29753156/antiviral-t-cells-for-adenovirus-in-the-pre-transplant-period-a-bridge-therapy-for-severe-combined-immunodeficiency
#14
Holly K Miller, Patrick J Hanley, Haili Lang, Christopher A Lazarski, Elizabeth A Chorvinsky, Sarah McCormack, Lauren Roesch, Shuroug Albihani, Marcus Dean, Fahmida Hoq, Roberta H Adams, Catherine Bollard, Michael D Keller
Viral infections can be life-threatening in patients with severe combined immunodeficiency (SCID) and other forms of profound primary immunodeficiency disorders both before and after hematopoietic stem cell transplant (HSCT). Adoptive immunotherapy with virus-specific T-cells (VST) has been utilized in many patients in the setting of HSCT, but has very rarely been attempted for treatment of viral infections before HSCT. Here we describe the use of VSTs in an infant with RAG1 SCID who had developed disseminated adenovirus which failed to improve on cidofovir...
May 9, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29752681/critical-role-of-beclin1-in-hiv-tat-and-morphine-induced-inflammation-and-calcium-release-in-glial-cells-from-autophagy-deficient-mouse
#15
Jessica Lapierre, Myosotys Rodriguez, Chet Raj Ojha, Nazira El-Hage
We previously showed that autophagy is an important component in human immunodeficiency virus (HIV) replication and in the combined morphine-induced neuroinflammation in human astrocytes and microglia. Here we further studied the consequences of autophagy using glial cells of mice partially lacking the essential autophagy gene Atg6 (Beclin1) exposed to HIV Tat and morphine. Tat is known to cause an inflammatory response, increase calcium release, and possibly interact with autophagy pathway proteins. Following Tat exposure, autophagy-deficient (Becn1+/- ) glial cells had significantly and consistently reduced levels in the pro-inflammatory cytokine IL-6 and the chemokines RANTES and MCP-1 when compared to Tat-treated cells from control (C57BL/6J) mice, suggesting an association between the inflammatory effects of Tat and Beclin1...
May 11, 2018: Journal of Neuroimmune Pharmacology: the Official Journal of the Society on NeuroImmune Pharmacology
https://www.readbyqxmd.com/read/29750890/impact-of-age-on-disease-progression-and-microenvironment-in-oral-cancer
#16
V K Vincent-Chong, H DeJong, L J Rich, A Patti, M Merzianu, P A Hershberger, M Seshadri
Despite the recognized link between aging and cancer, most preclinical studies in experimental tumor models are conducted with 6- to 8-wk-old rodents. The goal of the present study was to examine the impact of age on tumor incidence, growth, and microenvironmental characteristics in mouse models of head and neck squamous cell carcinoma (HNSCC). Experimental studies were conducted with the 4-nitroquinoline-oxide (4NQO) oral carcinogenesis model and orthotopic FaDu HNSCC xenografts, established in young (7 to 12 wk of age) and old (65 to 70 wk of age) female C57BL/6 mice ( n = 44; 4NQO model) and severe combined immunodeficient mice ( n = 13; HNSCC xenografts)...
May 1, 2018: Journal of Dental Research
https://www.readbyqxmd.com/read/29750804/temporal-compartmental-changes-in-viral-rna-and-neuronal-injury-in-a-primate-model-of-neuroaids
#17
R Gilberto González, Robert Fell, Julian He, Jennifer Campbell, Tricia H Burdo, Patrick Autissier, Lakshmanan Annamalai, Faramarz Taheri, Termara Parker, Jeffrey D Lifson, Elkan F Halpern, Mark Vangel, Eliezer Masliah, Susan V Westmoreland, Kenneth C Williams, Eva-Maria Ratai
Despite the advent of highly active anti-retroviral therapy HIV-associated neurocognitive disorders (HAND) continue to be a significant problem. Furthermore, the precise pathogenesis of this neurodegeneration is still unclear. The objective of this study was to examine the relationship between infection by the simian immunodeficiency virus (SIV) and neuronal injury in the rhesus macaque using in vivo and postmortem sampling techniques. The effect of SIV infection in 23 adult rhesus macaques was investigated using an accelerated NeuroAIDS model...
2018: PloS One
https://www.readbyqxmd.com/read/29750285/updates-in-newborn-screening
#18
Farrah Rajabi
Newborn screening in the United States is an important public health measure to provide early detection for specified disorders when early treatment is both possible and beneficial. As technology improves, newborn screening can be offered for many more conditions. In the past 10 years, screening has expanded to include severe combined immunodeficiency, congenital heart disease, lysosomal storage disease, and X-linked adrenoleukodystrophy. This article reviews the current state of newborn screening with updates on recent developments...
May 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29749587/newborn-screening-for-severe-combined-immunodeficiency
#19
REVIEW
John Routes, James Verbsky
PURPOSE OF REVIEW: This review provides a brief history of newborn screening (NBS) for severe combined immunodeficiency (SCID), discusses the theoretical basis for the T cell receptor excision circle (TREC) assay, highlights the results of recent studies using the TREC, and provides practical advice for the evaluation of infants with an abnormal TREC assay. RECENT FINDINGS: Currently, all but three states perform NBS for SCID in the USA. NBS using the TREC assay is highly sensitive in identifying infants with SCID and may also identify infants with T cell lymphopenia due to other causes such as congenital syndromes, multiple congenital anamolies, and some combined immunodeficiencies...
May 10, 2018: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/29749435/spag6-silencing-induces-apoptosis-in-the-myelodysplastic-syndrome-cell-line-skm%C3%A2-1-via-the-pten-pi3k-akt-signaling-pathway-in-vitro-and-in-vivo
#20
Jiaxiu Yin, Xinxin Li, Zaili Zhang, Xiaohua Luo, Li Wang, Lin Liu
Apoptosis is a multi-step mechanism of cell self‑destruction for maintaining cellular homeostatic balance. Accumulating evidence indicates that abnormal apoptosis promotes the evolution and progression of myelodysplastic syndromes (MDS). As a novel cancer-testis antigen, sperm‑associated antigen 6 (SPAG6) has been reported to regulate apoptosis through the tumor necrosis factor-related apoptosis-inducing ligand signaling pathway in the MDS cell line SKM‑1. However, the mechanism of the intrinsic cell death pathway for apoptosis induction by SPAG6 silencing is unclear...
May 2, 2018: International Journal of Oncology
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