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severe combined immune deficiency

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https://www.readbyqxmd.com/read/28639520/polyomavirus-associated-prostatitis-in-wistar-han-rats-following-immunosuppression-in-a-chronic-toxicity-study
#1
Katherine Masek-Hammerman, Thomas P Brown, Walter F Bobrowski, Lindsay Tomlinson, Marie Debrue, Laurence Whiteley, Zaher Radi
Chronic prostatitis characterized on light microscopic examination by moderate, multifocal, predominantly lymphocytic inflammation associated with epithelial atypia and intranuclear and cytoplasmic inclusion-like material was identified in the prostate gland of 2 Wistar Han rats administered an immunomodulatory test article in a 6-month chronic toxicity study. Transmission electron microscopy of the prostate glands identified 45-nm, nonenveloped, icosahedral virions arranged in paracrystalline array within the cell nuclei in 1 of the 2 rats...
January 1, 2017: Toxicologic Pathology
https://www.readbyqxmd.com/read/28634034/absence-of-%C3%AE-chain-in-keratinocytes-alters-chemokine-secretion-resulting-in-reduced-immune-cell-recruitment
#2
Karolin Nowak, Daniela Linzner, Adrian J Thrasher, Paul F Lambert, Wei Li Di, Siobhan O Burns
Loss of function mutations in the common gamma (γc) chain cytokine receptor subunit give rise to severe combined immunodeficiency (SCID) characterised by lack of T and natural killer cells and infant death from infection. Haematopoietic stem cell transplantation or gene therapy offer cure but despite successful replacement of lymphoid immune lineages a long-term risk of severe cutaneous human papilloma virus (HPV) infections persists, possibly related to persistent γc-deficiency in other cell types. Here we demonstrate that keratinocytes, the only cell type directly infected by HPV, express functional γc and its co-receptors...
June 17, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28628017/-combined-immunotherapy-of-reconditional-chronic-non-specific-vulvovaginitis-in-immunocompromised-girls
#3
I Nesterova, S Kovaleva, G Chudilova, L Lomtatidze, V Krutova, I Aslanian, A Tulendinova, V Malinovskaya
Nonspecific chronic vulvovaginitis (CNV) is often a clinical indicator of immune deficiency, especially in young girls. The established violations of the functioning of various parts of the immune system (IS) in this pathology dictate the need to include in the complex of immunomodulatory therapy. The developed program of combined immunotherapy for immunocompromised girls allows to reduce the severity and duration of exacerbation of CNV, their frequency against the background of a significant reduction in the incidence of ARVI...
May 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28626409/hiv-associated-cerebellar-dysfunction-and-improvement-with-aminopyridine-therapy-a-case-report
#4
Carolin Hoyer, Angelika Alonso, Beate Schlotter-Weigel, Michael Platten, Marc Fatar
Apart from infectious causes and cerebellar dysfunction associated with acquired immune deficiency syndrome dementia or HIV-associated neurocognitive disorder, cerebellar dysfunction in HIV-positive individuals has been ascribed to granule cell neuronopathy as well as primary cerebellar atrophy without identifiable etiology. We report the case of a patient with progressive cerebellar dysfunction as the primary manifestation of HIV infection. No symptom improvement was seen under combination antiretroviral therapy, which had been established upon diagnosis, but the patient improved rapidly under 4-aminopyridine treatment, which was recommended 1 year later...
May 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28567065/the-psychoneuroendocrine-immunotherapy-of-cancer-historical-evolution-and-clinical-results
#5
REVIEW
Paolo Lissoni, Giusy Messina, Arianna Lissoni, Rovelli Franco
The prognosis of the neoplastic diseases depends not only on the biogenetic characteristics of cancer cells but also on the immunological response of patients, which may influence the biological features of cancer cells themselves as well as the angiogenic processes. Moreover, the immune system in vivo is under a physiological psychoneuroendocrine (PNE) regulation, mainly mediated by the brain opioid system and the pineal gland. In more detail, the anticancer immunity is stimulated by the pineal hormone melatonin (MLT) and inhibited by the opioid system, namely, through a mu-opioid receptor...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28555324/low-maternal-circulating-levels-of-vitamin-d-as-potential-determinant-in-the-development-of-gestational-diabetes-mellitus
#6
REVIEW
S Triunfo, A Lanzone, P G Lindqvist
Gestational diabetes mellitus (GDM), defined as any glucose intolerance with the onset or first recognition during pregnancy, is characterized by rising incidence, fostered by the worldwide increase of pathological nutritional status from young age. Clinical research has intended to identify potential risk factors, suggested improvements in screening strategies, and recommended the combination between promotion of an appropriate lifestyle before and during pregnancy and selected therapeutic approaches. Preventing pathological hyperglycemia could have several benefits, ranging from clinical side (reduction in the risk of adverse perinatal and long-term sequelae) to financial side (cost reduction to healthcare systems)...
May 29, 2017: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/28527810/hif-1%C3%AE-stabilizing-agent-fg-4497-rescues-human-cd34-cell-mobilization-in%C3%A2-response-to-g-csf-in-immunodeficient-mice
#7
Bianca Nowlan, Kathryn Futrega, Marion E Brunck, Gail Walkinshaw, Lee E Flippin, Michael R Doran, Jean-Pierre Levesque
Granulocyte colony-stimulating factor (G-CSF) is used routinely in the clinical setting to mobilize hematopoietic stem progenitor cells (HSPCs) into the patient's blood for collection and subsequent transplantation. However, a significant proportion of patients who have previously received chemotherapy or radiotherapy and require autologous HSPC transplantation cannot mobilize the minimal threshold of mobilized HSPCs to achieve rapid and successful hematopoietic reconstitution. Although several alternatives to the G-CSF regime have been tested, few are used in the clinical setting...
May 17, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28513593/jak3-deficiency-blocks-innate-lymphoid-cell-development
#8
M L Robinette, M Cella, J B Telliez, T K Ulland, A D Barrow, K Capuder, S Gilfillan, L-L Lin, L D Notarangelo, M Colonna
Loss-of-function mutations in the tyrosine kinase JAK3 cause autosomal recessive severe combined immunodeficiency (SCID). Defects in this form of SCID are restricted to the immune system, which led to the development of immunosuppressive JAK inhibitors. We find that the B6.Cg-Nr1d1(tm1Ven)/LazJ mouse line purchased from Jackson Laboratories harbors a spontaneous mutation in Jak3, generating a SCID phenotype and an inability to generate antigen-independent professional cytokine-producing innate lymphoid cells (ILCs)...
May 17, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28499891/sapho-autophagy-il-1-foxo1-and-propionibacterium-cutibacterium-acnes
#9
Jean-Marie Berthelot, Stéphane Corvec, Gilles Hayem
Overt infection by Propionibacterium acnes is lacking in many SAPHO syndromes, and antibiotics have only a transient and incomplete effect, either in SAPHO syndrome or acne. As several auto-inflammatory bone disorders sharing overproduction of IL-1β can mimic SAPHO, this syndrome could partly depend on genetically encoded overproduction of IL-1β. However, cyclic intracellular infections, mostly by P. acnes, can contribute to the enhanced IL-1β release by some skin cells, and probably by bone cells. P. acnes is indeed a powerful trigger of NLRP3-inflammasome activation and IL-1β, leading to osteitis and enhanced mesenchymal cells differentiation in osteoblasts...
May 9, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28480786/ultraviolet-a1-irradiation-therapy-for-systemic-lupus-erythematosus
#10
H McGrath
Systemic lupus erythematosus (lupus, SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, which bind to antigens and are deposited within tissues to fix complement, resulting in widespread systemic inflammation. The studies presented herein are consistent with hyperpolarized, adenosine triphosphate (ATP)-deficient mitochondria being central to the disease process. These hyperpolarized mitochondria resist the depolarization required for activation-induced apoptosis. The mitochondrial ATP deficits add to this resistance to apoptosis and also reduce the macrophage energy that is needed to clear apoptotic bodies...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28446609/glycosylation-of-the-core-of-the-hiv-1-envelope-subunit-protein-gp120-is-not-required-for-native-trimer-formation-or-viral-infectivity
#11
Ujjwal Rathore, Piyali Saha, Sannula Kesavardhana, Aditya Arun Kumar, Rohini Datta, Sivasankar Devanarayanan, Raksha Das, John R Mascola, Raghavan Varadarajan
The gp120 subunit of the HIV-1 envelope (Env) protein is heavily glycosylated at ∼25 glycosylation sites, of which ∼7-8 are located in the V1/V2 and V3 variable loops and the others in the remaining core gp120 region. Glycans partially shield Env from recognition by the host immune system and also are believed to be indispensable for proper folding of gp120 and for viral infectivity. Previous attempts to alter glycosylation sites in Env typically involved mutating the glycosylated asparagine residues to structurally similar glutamines or alanines...
June 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28410768/-small-fiber-neuropathy
#12
V Langlois, A-L Bedat Millet, M Lebesnerais, S Miranda, F Marguet, Y Benhamou, P Marcorelles, H Lévesque
Small fiber neuropathy (SFN) is still unknown. Characterised by neuropathic pain, it typically begins by burning feet, but could take many other expression. SFN affects the thinly myelinated Aδ and unmyelinated C-fibers, by an inherited or acquired mechanism, which could lead to paresthesia, thermoalgic disorder or autonomic dysfunction. Recent studies suggest the preponderant role of ion channels such as Nav1.7. Furthermore, erythromelalgia or burning mouth syndrome are now recognized as real SFN. Various aetiologies of SFN are described...
April 11, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28390676/nitric-oxide-donor-augments-antineoplastic-effects-of-arginine-deprivation-in-human-melanoma-cells
#13
Oksana Mayevska, Oleh Chen, Olena Karatsai, Yaroslav Bobak, Maryna Barska, Liliana Lyniv, Iuliia Pavlyk, Yuriy Rzhepetskyy, Natalia Igumentseva, Maria Jolanta Redowicz, Oleh Stasyk
Anticancer therapy based on recombinant arginine-degrading enzymes has been proposed for the treatment of several types of malignant cells deficient in arginine biosynthesis. One of the predicted side effects of such therapy is restricted bioavailability of nitric oxide as arginine catabolic product. Prolonged NO limitation may lead to unwanted disturbances in NO-dependent vasodilation, cardiovascular and immune systems. This problem can be overcome by co-supplementation with exogenous NO donor. However, NO may potentially counteract anticancer effects of therapy based on arginine deprivation...
April 5, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28381283/consolidating-emerging-evidence-surrounding-hivst-and-hivss-a-rapid-systematic-mapping-protocol
#14
T Charles Witzel, Peter Weatherburn, Fiona M Burns, Cheryl C Johnson, Carmen Figueroa, Alison J Rodger
BACKGROUND: HIV self-testing (HIVST) is becoming popular with policy makers and commissioners globally, with a key aim of expanding access through reducing barriers to testing for individuals at risk of HIV infection. HIV self-sampling (HIVSS) was available previously to self-testing but was confined mainly to the USA and the UK. It remains to be seen whether the momentum behind HIVST will also energise efforts to expand HIVSS. Recent years have seen a rapid growth in the type of evidence related to these interventions as well as several systematic reviews...
April 5, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28378771/mediation-of-transitional-b-cell-maturation-in-the-absence-of-functional-bruton-s-tyrosine-kinase
#15
Shalini Tanwar, Atika Dhar, Vineeth Varanasi, Tapas Mukherjee, Ramanamurthy Boppana, Soumen Basak, Vineeta Bal, Anna George, Satyajit Rath
X-linked immune-deficient (Xid) mice, carrying a mutation in Bruton's tyrosine kinase (Btk), have multiple B cell lineage differentiation defects. We now show that, while Xid mice showed only mild reduction in the frequency of the late transitional (T2) stage of peripheral B cells, the defect became severe when the Xid genotype was combined with either a CD40-null, a TCRbeta-null or an MHC class II (MHCII)-null genotype. Purified Xid T1 and T2 B cells survived poorly in vitro compared to wild-type (WT) cells...
April 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28367508/alterations-of-the-murine-gut-microbiome-in-allergic-airway-disease-are-independent-of-surfactant-protein-d
#16
Kenneth K Barfod, Michael Roggenbuck, Suzan Al-Shuweli, Dalia Fakih, Søren J Sørensen, Grith L Sørensen
BACKGROUND: SP-D is an important host defense lectin in innate immunity and SP-D deficient mice show several abnormal immune effects and are susceptible to allergen-induced airway disease. At the same time, host microbiome interactions play an important role in the development of allergic airway disease, and alterations to gut microbiota have been linked to airway disease through the gut-lung axis. Currently, it is unknown if the genotype (Sftpd-/- or Sftpd+/+) of the standard SP-D mouse model can affect the host microbiota to such an degree that it would overcome the cohousing effect on microbiota and interfere with the interpretation of immunological data from the model...
March 2017: Heliyon
https://www.readbyqxmd.com/read/28363996/mir-34a-and-mir-34b-c-suppress-intestinal-tumorigenesis
#17
Longchang Jiang, Heiko Hermeking
The p53-inducible miR-34a and miR-34b/c genes are frequently silenced in colorectal cancer. To address the in vivo relevance of miR-34a/b/c function for suppression of intestinal tumor formation, we generated Apc(Min/+) mice with deletions of the miR-34a and/or miR-34b/c genes separately or in combination. Combined deletion of miR-34a/b/c increased the number of intestinal stem cells as well as Paneth and Goblet cells, resulting in enlarged intestinal crypts. miR-34a/b/c-deficient Apc(Min/+) mice displayed an increased tumor burden and grade and decreased survival...
May 15, 2017: Cancer Research
https://www.readbyqxmd.com/read/28330936/biochemical-principles-and-functional-aspects-of-pipecolic-acid-biosynthesis-in-plant-immunity
#18
Michael Hartmann, Denis Kim, Friederike Bernsdorff, Ziba Ajami-Rashidi, Nicola Scholten, Stefan Schreiber, Tatyana Zeier, Stefan Schuck, Vanessa Reichel-Deland, Jürgen Zeier
The nonprotein amino acid pipecolic acid (Pip) regulates plant systemic acquired resistance and basal immunity to bacterial pathogen infection. In Arabidopsis (Arabidopsis thaliana), the lysine (Lys) aminotransferase AGD2-LIKE DEFENSE RESPONSE PROTEIN1 (ALD1) mediates the pathogen-induced accumulation of Pip in inoculated and distal leaf tissue. Here, we show that ALD1 transfers the α-amino group of l-Lys to acceptor oxoacids. Combined mass spectrometric and infrared spectroscopic analyses of in vitro assays and plant extracts indicate that the final product of the ALD1-catalyzed reaction is enaminic 2,3-dehydropipecolic acid (DP), whose formation involves consecutive transamination, cyclization, and isomerization steps...
May 2017: Plant Physiology
https://www.readbyqxmd.com/read/28325287/cholesterol-containing-nuclease-resistant-sirna-accumulates-in-tumors-in-a-carrier-free-mode-and-silences-mdr1-gene
#19
Ivan V Chernikov, Daniil V Gladkikh, Mariya I Meschaninova, Alya G Ven'yaminova, Marina A Zenkova, Valentin V Vlassov, Elena L Chernolovskaya
Chemical modifications are an effective way to improve the therapeutic properties of small interfering RNAs (siRNAs), making them more resistant to degradation in serum and ensuring their delivery to target cells and tissues. Here, we studied the carrier-free biodistribution and biological activity of a nuclease-resistant anti-MDR1 cholesterol-siRNA conjugate in healthy and tumor-bearing severe combined immune deficiency (SCID) mice. The attachment of cholesterol to siRNA provided its efficient accumulation in the liver and in tumors, and reduced its retention in the kidneys after intravenous and intraperitoneal injection...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28280951/effects-of-dietary-n-3-fatty-acids-on-toll-like-receptor-activation-in-primary-leucocytes-from-atlantic-salmon-salmo-salar
#20
Marianne Arnemo, Arturas Kavaliauskis, Adriana Magalhaes Santos Andresen, Marta Bou, Gerd Marit Berge, Bente Ruyter, Tor Gjøen
The shortage of the n-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) on the international markets has led to increasing substitution of fish oil by plant oils in Atlantic salmon (Salmo salar) feed and thereby reducing the EPA and DHA content in salmon. However, the minimum required levels of these fatty acids in fish diets for securing fish health are unknown. Fish were fed with 0, 1 or 2% EPA or DHA alone or in combination of both over a period, growing from 50 to 400 g. Primary head kidney leucocytes were isolated and stimulated with Toll-like receptor (TLR) ligands to determine if EPA and DHA deficiency can affect expression of important immune genes and eicosanoid production...
March 9, 2017: Fish Physiology and Biochemistry
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