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Primary immune deficiency

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https://www.readbyqxmd.com/read/29147012/piperidylmethyloxychalcone-improves-immune-mediated-acute-liver-failure-via-inhibiting-tak1-activity
#1
Sun Hong Park, Jeong-Ah Kwak, Sang-Hun Jung, Byeongwoo Ahn, Won-Jea Cho, Cheong-Yong Yun, Chang Seon Na, Bang Yeon Hwang, Jin Tae Hong, Sang-Bae Han, Youngsoo Kim
Mice deficient in the toll-like receptor (TLR) or the myeloid differentiation factor 88 (MyD88) are resistant to acute liver failure (ALF) with sudden death of hepatocytes. Chalcone derivatives from medicinal plants protect from hepatic damages including ALF, but their mechanisms remain to be clarified. Here, we focused on molecular basis of piperidylmethyloxychalcone (PMOC) in the treatment of TLR/MyD88-associated ALF. C57BL/6J mice were sensitized with D-galactosamine (GalN) and challenged with Escherichia coli lipopolysaccharide (LPS, TLR4 agonist) or oligodeoxynucleotide containing unmethylated CpG motif (CpG ODN, TLR9 agonist) for induction of ALF...
November 17, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29140126/new-insights-into-the-role-of-vitamin-d-in-hepatocellular-carcinoma
#2
Dong-Bo Wu, Meng-Lan Wang, En-Qiang Chen, Hong Tang
In addition to being crucial for host immune defense, vitamin D is involved in cell proliferation, apoptosis, differentiation, inflammation, invasion and metastasis, angiogenesis and micro-RNA modulation. To date, clinical studies have demonstrated that vitamin D deficiency is common not only in patients with chronic liver diseases but also in those with hepatocellular carcinoma (HCC). Experimental studies have also demonstrated that vitamin D and its receptors are related to the occurrence of HCC and the prognoses of patients with HCC...
November 15, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29139565/leukocyte-adhesion-defect-i-rare-primary-immune-deficiency
#3
Nitesh Tewari, Vijay Prakash Mathur, Vikender Singh Yadav, Prabhat Chaudhari
Leukocyte adhesion defect I is a rare disorder (1:1,000,000) caused by diminished expression of CD-18 β2 integrins on leukocytes leading to abnormal adhesion, migration, and chemotaxis. Clinical manifestations include delayed separation of umbilical cord, omphalitis, recurrent severe infections, impaired wound healing, persistent oral ulcers, and severe periodontitis in primary and permanent dentition. A 5-year-old girl, second-born child to parents with consanguinity, presented with pain and mobility of lower teeth...
November 15, 2017: Special Care in Dentistry
https://www.readbyqxmd.com/read/29138299/induction-of-antiviral-immune-response-through-recognition-of-the-repeating-subunit-pattern-of-viral-capsids-is-toll-like-receptor-2-dependent
#4
Kelly M Shepardson, Benjamin Schwarz, Kyle Larson, Rachelle V Morton, John Avera, Kimberly McCoy, Alayna Caffrey, Ann Harmsen, Trevor Douglas, Agnieszka Rynda-Apple
Although viruses and viral capsids induce rapid immune responses, little is known about viral pathogen-associated molecular patterns (PAMPs) that are exhibited on their surface. Here, we demonstrate that the repeating protein subunit pattern common to most virus capsids is a molecular pattern that induces a Toll-like-receptor-2 (TLR2)-dependent antiviral immune response. This early antiviral immune response regulates the clearance of subsequent bacterial superinfections, which are a primary cause of morbidities associated with influenza virus infections...
November 14, 2017: MBio
https://www.readbyqxmd.com/read/29136637/transcriptional-profiling-of-toll-like-receptor-2-deficient-primary-murine-brain-cells-during-toxoplasma-gondii-infection
#5
Kousuke Umeda, Sachi Tanaka, Fumiaki Ihara, Junya Yamagishi, Yutaka Suzuki, Yoshifumi Nishikawa
BACKGROUND: Toxoplasma gondii is capable of persisting in the brain, although it is efficiently eliminated by cellular immune responses in most other sites. While Toll-like receptor 2 (TLR2) reportedly plays important roles in protective immunity against the parasite, the relationship between neurological disorders induced by T. gondii infection and TLR2 function in the brain remains controversial with many unknowns. In this study, primary cultured astrocytes, microglia, neurons, and peritoneal macrophages obtained from wild-type and TLR2-deficient mice were exposed to T...
2017: PloS One
https://www.readbyqxmd.com/read/29132376/ezh2-suppression-in-glioblastoma-shifts-microglia-toward-m1-phenotype-in-tumor-microenvironment
#6
Yatao Yin, Shuwei Qiu, Xiangpen Li, Bo Huang, Yun Xu, Ying Peng
BACKGROUND: Glioblastoma multiforme (GBM) induces tumor immunosuppression through interacting with tumor-infiltrating microglia or macrophages (TAMs) with an unclear pathogenesis. Enhancer of zeste homolog 2 (EZH2) is abundant in GBM samples and cell lines and is involved in GBM proliferation, cell cycle, and invasion, whereas its association with innate immune response is not yet reported. Herein, the aim of this study was to investigate the role of EZH2 in GBM immune. METHODS: Co-culturing models of human/murine GBM cells with PBMC-derived macrophages/primary microglia were employed...
November 13, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29131033/adverse-reactions-to-biologics-in-psoriasis
#7
Stephen J Lockwood, Lisette M Prens, Alexa B Kimball
Psoriasis is a chronic autoimmune disease which affects millions of people worldwide. Not only can psoriasis itself be debilitating and significantly reduce an individual's quality of life, but it is also a risk factor for other systemic disorders, such as metabolic syndrome, cardiovascular disease, and malignancy. Tremendous strides were made in the treatment of psoriasis during the mid-to-late-20th century, including the emergence of topical corticosteroids and vitamin D analogs, methotrexate, systemic retinoids, and phototherapy...
2018: Current Problems in Dermatology
https://www.readbyqxmd.com/read/29128761/inborn-errors-of-immunity-underlying-fungal-diseases-in-otherwise-healthy-individuals
#8
REVIEW
Juan Li, Donald C Vinh, Jean-Laurent Casanova, Anne Puel
It has been estimated that there are at least 1.5 million fungal species, mostly present in the environment, but only a few of these fungi cause human disease. Most fungal diseases are self-healing and benign, but some are chronic or life-threatening. Acquired and inherited defects of immunity, including breaches of mucocutaneous barriers and circulating leukocyte deficiencies, account for most severe modern-day mycoses. Other types of infection typically accompany these fungal infections. More rarely, severe fungal diseases can strike otherwise healthy individuals...
November 9, 2017: Current Opinion in Microbiology
https://www.readbyqxmd.com/read/29128406/recognition-of-candida-albicans-by-gingival-fibroblasts-the-role-of-tlr2-tlr4-cd14-and-myd88
#9
Claudia Ramos Pinheiro, Ana Lúcia Coelho, Carine Ervolino de Oliveira, Thaís Helena Gasparoto, Gustavo Pompermaier Garlet, João Santana Silva, Carlos Ferreira Santos, Karen Angélica Cavassani, Cory M Hogaboam, Ana Paula Campanelli
Recent evidence indicates that nonprofessional immune cells such as epithelial cells, endothelial cells, and fibroblasts also contribute to innate immunity via secretion of cytokines. Fibroblasts are the principal type of cell found in the periodontal connective tissues and they are involved in the immune response during periodontal disease. The role of fibroblasts in the recognition of pathogens via Toll-like receptors (TLRs) has been established; however, few studies have been conducted concerning the involvement of innate immune receptors in the recognition of Candida albicans by gingival fibroblast...
November 8, 2017: Cytokine
https://www.readbyqxmd.com/read/29127199/the-full-length-interleukin-33-flil33-importin-5-interaction-does-not-regulate-flil33-s-nuclear-localization-but-controls-its-intracellular-degradation
#10
Andrew Clerman, Zahid Noor, Rita Fishelevich, Virginia Lockatell, Brian S Hampton, Nirav G Shah, Mariah V Salcedo, Nevins W Todd, Sergei P Atamas, Irina G Luzina
Human mature interleukin-33 (MIL33) is a member of the IL-1 family and a potent regulator of immunity through its pro-T helper cell 2 (Th2) activity. Its precursor form, full-length interleukin-33 (FLIL33), is an intranuclear protein in many cell types, including fibroblasts, and its intracellular levels can change in response to stimuli. However, the mechanisms controlling the nuclear localization of FLIL33 or its stability in cells are not understood. Here, we identified importin-5 (IPO5), a member of the importin family of nuclear transport proteins, as an intracellular binding partner of FLIL33...
November 10, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29126302/the-clinical-utility-of-measuring-igg-subclass-immunoglobulins-during-immunological-investigation-for-suspected-primary-antibody-deficiencies
#11
Antony R Parker, Markus Skold, David B Ramsden, J Gonzalo Ocejo-Vinyals, Marcos López-Hoyos, Stephen Harding
Measurement of IgG subclass concentrations is a standard laboratory test run as part of a panel to investigate the suspicion of antibody deficiency. The assessment is clinically important when total IgG is within the normal age-specific reference range. The measurement is useful for diagnosis of IgG subclass deficiency, to aid the diagnosis of specific antibody deficiency, as a supporting test for the diagnosis of common variable immunodeficiency, as well as for risk stratification of patients with low IgA...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29119939/mannose-binding-lectin-protein-deficiency-among-patients-with-primary-immunodeficiency-disease-receiving-ivig-therapy
#12
Gholamreza Azizi, Fatemeh Kiaee, Naeimeh Tavakolinia, Hosein Rafiemanesh, Sara Mohammadikhajehdehi, Laleh Sharifi, Reza Yazdani, Hassan Abolhassani, Asghar Aghamohammadi
BACKGROUND: Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of the immune system are defective. Immunoglobulin replacement therapy is the mainstay of treatment for patients with impaired antibody production. However, recurrent infections would continue to occur in some patients due to the other high frequent concomitant defects such as mannose-binding lectin (MBL) deficiency. METHODS: A total of 51 PID patients participated in this cross-sectional study...
November 7, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/29118090/complement-activation-via-a-c3a-receptor-pathway-alters-cd4-t-lymphocytes-and-mediates-lung-cancer-progression
#13
Jeff W Kwak, Jennifer Laskowski, Howard Y Li, Maria V McSharry, Trisha R Sippel, Bonnie L Bullock, Amber M Johnson, Joanna M Poczobutt, Alexander J Neuwelt, Stephen P Malkoski, Mary C Weiser-Evans, John Lambris, Eric T Clambey, Joshua M Thurman, Raphael A Nemenoff
The complement cascade is a part of the innate immune system which acts primarily to remove pathogens and injured cells. However, complement activation is also peculiarly associated with tumor progression. Here we report mechanistic insights into this association in multiple immunocompetent orthotopic models of lung cancer. After tumor engraftment, we observed systemic activation of the complement cascade as reflected by elevated levels of the key regulator C3a. Notably, growth of primary tumors and metastases was both strongly inhibited in C3-deficient mice (C3-/- mice), with tumors undetectable in many subjects...
November 8, 2017: Cancer Research
https://www.readbyqxmd.com/read/29103428/overview-of-inflammation-in-neurometabolic-diseases
#14
Gregory A Grabowski
Neuroinflammation is an intrinsic component of the neurodegeneration of inborn errors of neurometabolic diseases. Diseases resulting in lysosomal, peroxisomal, and autophagocytic disruption lead to neuroinflammation by different mechanisms relating to accumulated substrates and/or downstream deficiencies that cause presymptomatic microglial activation, axonal instabilities and/or direct hyperactivation of intrinsic inflammatory mechanisms. Only in selected diseases is the blood-brain barrier (BBB) breached, thereby permitting peripheral adaptive immune mechanisms to amplify intrinsic immune reactions in the central nervous system...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29102562/prostaglandin-e2-increases-lentiviral-vector-transduction-efficiency-of-adult-human-hematopoietic-stem-and-progenitor-cells
#15
Garrett C Heffner, Melissa Bonner, Lauryn Christiansen, Francis J Pierciey, Dakota Campbell, Yegor Smurnyy, Wenliang Zhang, Amanda Hamel, Seema Shaw, Gretchen Lewis, Kendrick A Goss, Olivia Garijo, Bruce E Torbett, Holly Horton, Mitchell H Finer, Philip D Gregory, Gabor Veres
Gene therapy currently in development for hemoglobinopathies utilizes ex vivo lentiviral transduction of CD34(+) hematopoietic stem and progenitor cells (HSPCs). A small-molecule screen identified prostaglandin E2 (PGE2) as a positive mediator of lentiviral transduction of CD34(+) cells. Supplementation with PGE2 increased lentiviral vector (LVV) transduction of CD34(+) cells approximately 2-fold compared to control transduction methods with no effect on cell viability. Transduction efficiency was consistently increased in primary CD34(+) cells from multiple normal human donors and from patients with β-thalassemia or sickle cell disease...
October 5, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29101209/oral-epithelial-cells-orchestrate-innate-type-17-responses-to-candida-albicans-through-the-virulence-factor-candidalysin
#16
Akash H Verma, Jonathan P Richardson, Chunsheng Zhou, Bianca M Coleman, David L Moyes, Jemima Ho, Anna R Huppler, Kritika Ramani, Mandy J McGeachy, Ilgiz A Mufazalov, Ari Waisman, Lawrence P Kane, Partha S Biswas, Bernhard Hube, Julian R Naglik, Sarah L Gaffen
Candida albicans is a dimorphic commensal fungus that causes severe oral infections in immunodeficient patients. Invasion of C. albicans hyphae into oral epithelium is an essential virulence trait. Interleukin-17 (IL-17) signaling is required for both innate and adaptive immunity to C. albicans During the innate response, IL-17 is produced by γδ T cells and a poorly understood population of innate-acting CD4(+) αβ T cell receptor (TCRαβ)(+) cells, but only the TCRαβ(+) cells expand during acute infection...
November 3, 2017: Science Immunology
https://www.readbyqxmd.com/read/29095280/primary-hypothyroidism-and-isolated-acth-deficiency-induced-by-nivolumab-therapy-case-report-and-review
#17
REVIEW
Mei Fang Zeng, Li Chen, Hong Ying Ye, Wei Gong, Li Nuo Zhou, Yi Ming Li, Xiao Long Zhao
RATIONALE: Nivolumab is a monoclonal IgG antibody blocking programmed death receptor-1 (PD1), leading to restoration of the natural T-cell-mediated immune response against the cancer cells. However, it also causes plenty of autoimmune-related adverse events, which often involves endocrine system. PATIENT CONCERNS: A 54-year-old male with renal clear cell carcinoma was treated with nivolumab intravenously. Routine monitoring showed elevated thyroid-stimulating hormone and low free thyroxine after the 6th administration of nivolumab...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29094026/establishing-a-colorectal-cancer-liver-metastasis-patient-derived-tumor-xenograft-model-for-the-evaluation-of-personalized-chemotherapy
#18
Joohee Jung, Jisup Kim, Hyun Kyung Lim, Kyoung Mee Kim, Yun Sun Lee, Joon Seong Park, Dong Sup Yoon
Purpose: In order to suggest optimal anticancer drugs for patient-tailored chemotherapy, we developed a colorectal cancer (CRC)-liver metastasis patient-derived tumor xenograft (PDTX) model. Methods: Tissue obtained from a patient with CRC-liver metastasis (F0) was transplanted in a nonobese female mouse with diabetic/severe combined immune deficiency (F1) and the tumor tissue was retransplanted into nude mice (F2). When tumor volumes reached ~500 mm(3), the F2 mice were randomly divided into 4 groups (n = 4/group) of doxorubicin, cisplatin, docetaxel, and nontreated control groups...
October 2017: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/29093656/functional-defects-in-type-3-innate-lymphoid-cells-and-classical-monocytes-in-a-patient-with-hyper-ige-syndrome
#19
Yuna Chang, Sung-Yoon Kang, Jihyun Kim, Hye-Ryun Kang, Hye Young Kim
Hyper-IgE syndrome (HIES) is a very rare primary immune deficiency characterized by elevated serum IgE levels, recurrent bacterial infections, chronic dermatitis, and connective tissue abnormalities. Autosomal dominant (AD) HIES involves a mutation in signal transducer and activator of transcription 3 (STAT3) that leads to an impaired TH17 response. STAT3 signaling is also involved in the function of RORγt(+) type 3 innate lymphoid cells (ILC3s) and RORγt(+)TH17 cells. The aim of this study was to investigate the role of innate immune cells such as innate lymphoid cells (ILCs), granulocytes, and monocytes in a patient with HIES...
October 2017: Immune Network
https://www.readbyqxmd.com/read/29091809/pharmacological-agents-preceding-a-diagnosis-of-immune-thrombocytopenia-in-adult-patients-developing-the-chronic-form-a-swedish-national-register-study
#20
Lukas Löfling, Marie Linder, Charlotta Ekstrand, Honar Cherif, Helle Kieler, Shahram Bahmanyar
Patients with primary chronic immune thrombocytopenia (cITP) have been reported to use more anti-infective medications, even before diagnosis of immune thrombocytopenia (ITP). The more common use of anti-infective medications may be due to general health problems, requiring medication in the early stages of cITP and before the diagnosis is set, rather than infections preceding the disease. Accordingly, cITP may not only be associated with use of anti-infective medications but also with medications for more general symptoms...
October 23, 2017: Thrombosis Research
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