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1.

001-es BibID:BIBFORM072048
Első szerző:Borrelli, M.
Cím:Intestinal anti-transglutaminase 2 immunoglobulin A deposits in children at risk for coeliac disease (CD) : data from the PreventCD study / Borrelli M., Maglio M., Korponay-Szabó I. R., Vass V., Mearin M. L., Meijer C., Niv-Drori H., Ribes-Koninckx C., Roca M., Shamir R., Troncone R., Auricchio R.
Dátum:2018
ISSN:0009-9104
Megjegyzések:In coeliac disease (CD), anti-tissue transglutaminase 2 immunoglobulin(Ig)A antibodies (anti-TG2) are produced and deposited in the intestine.PreventCD (www.preventcd.com) is a European multi-centre study, whichinvestigates the influence of infant nutrition and that of genetic,immunological and other environmental factors on the risk of developingCD. The aim of the current study was to evaluate the appearance ofintestinal anti-TG2 deposits in very early intestinal biopsies from at-riskinfants and their predictive value for villous atrophy. Sixty-five small bowelbiopsies, performed in 62 children, were investigated for the presenceof intestinal anti-TG2 extracellular IgA deposits by using doubleimmunofluorescence. The biopsies were performed in the presence ofelevated serum levels of CD-associated antibodies and/or symptomssuggesting disease. Deposits of anti-TG2 IgA were present in 53 of 53 CDpatients and three of three potential CD patients. In potential CD patients,mucosal deposits showed a patchy distribution characterized by some areascompletely negative, whereas active CD patients had uniformly present andevident mucosal deposits. Only one of six patients without CD (negative forserum anti-TG2 and with normal mucosa) had intestinal deposits with apatchy distribution and a weak staining. Two of the 53 CD patients receiveda definitive diagnosis of CD after a second or third biopsy; mucosal depositsof anti-TG2 IgA were evaluated in all samples. Before developing villous atrophy, both patients had anti-TG2 deposits in normal mucosalarchitecture, antibodies in one patient being absent in serum. We demonstrated that in CD the intestinal deposits of anti-TG2 are a constant presence and appear very early in the natural history of disease.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
celiac
Megjelenés:Clinical And Experimental Immunology. - 191 : 3 (2018), p. 311-317. -
További szerzők:Maglio, M. Korponay-Szabó Ilma (1959-) (gyermekgyógyász) Vass V. Mearin, Maria Luisa Meijer, Caroline R. Niv-Drori, H. Ribes-Koninckx, Carmen Roca, María Shamir, R. Troncone, Riccardo Auricchio, Renata
Pályázati támogatás:GINOP-2.3.2-15-2016-00015
GINOP
NKFI-120392
NKFI
OTKA-101788
OTKA
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2.

001-es BibID:BIBFORM099852
Első szerző:Erdős Melinda (infektológus, gyermekimmunológus)
Cím:Novel STAT-3 gain-of-function variant with hypogammaglobulinemia and recurrent infection phenotype / Erdős Melinda, Tsumura Miyuki, Kállai Judit, Lányi Árpád, Nyul Zoltán, Balázs György, Okada Satoshi, Maródi László
Dátum:2021
ISSN:0009-9104
Megjegyzések:Signal transducer and activator of transcription 3 (STAT3) gain-of-function (GOF) syndrome is an early-onset monogenic inborn error of immunity characterized by multi-organ autoimmune disorders, growth failure and lymphoproliferation. We describe that STAT3 GOF syndrome may be presented with hypogammaglobulinemia and recurrent severe upper and lower respiratory tract infections. In addition, the patient had lymphoproliferation, short stature and interstitial lung disease. Chest CT examinations showed mild bronchiectasis with areas of non-fibrosing alveolar-interstitial disease and maldevelopment of bilateral first ribs. By using Sanger sequencing, we revealed a novel c.508G>C, p.D170H STAT3 variant affecting the coiled coil domain of STAT3. Functional studies confirmed that p.D170H was a GOF variant as showed by increased pSTAT3 and STAT3 transcriptional activity. Our observation suggests that STAT3 GOF syndrome can manifest in early childhood with hypogammaglobulinemia and recurrent severe respiratory tract infections. We suggest that patients with lymphoproliferation, hypogammaglobulinemia and severe, recurrent infections should be screened for STAT3 variants even if autoimmune manifestations are missing.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
autoimmunity
gain-of-function
immune dysregulation
lymphoproliferative disease
short stature
STAT-3
Megjelenés:Clinical And Experimental Immunology. - 205 : 3 (2021), p. 354-362. -
További szerzők:Tsumura, Miyuki Kállai Judit (1983-) (molekuláris biológus) Lányi Árpád (1962-) (biológus, immunológus) Nyúl Zoltán Balázs György (1933-) (sebész) Okada, Satoshi Maródi László (1949-) (gyermekgyógyász infektológus, immunológus)
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DOI
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3.

001-es BibID:BIBFORM010677
Első szerző:Gathmann, B.
Cím:The European internet-based patient and research database for primary immunodeficiencies : results 2006-2008 / Gathmann B., Grimbacher B., Beauté J., Dudoit Y., Mahlaoui N., Fischer A., Knerr V., Kindle G., The ESID Registry Working Party
Dátum:2009
ISSN:0009-9104 (Print)
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical and Experimental Immunology. - 157 : Suppl.1 (2009), p. 3-11. -
További szerzők:Grimbacher, Bodo Beauté, J. Dudoit, Y. Mahlaoui, N. Fischer, A. Knerr, V. Kindle, Gerhard Maródi László (1949-) (gyermekgyógyász infektológus, immunológus) The ESID Registry Working Party
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4.

001-es BibID:BIBFORM035343
Első szerző:Holló Krisztina (vegyész)
Cím:Complex pattern of Th1 and Th2 activation with a preferential increase of autoreactive Th1 cells in BALB/c mice with proteoglycan (aggrecan)-induced arthritis / Holló K., Glant T. T., Garzó M., Finnegan A., Mikecz K., Buzás E.
Dátum:2000
ISSN:0009-9104
Megjegyzések:The central role of CD4+ T cells and the balance between T helper (Th) subpopulations in the pathogenesis of autoimmune diseases have been extensively studied. Proteoglycan (aggrecan)-induced arthritis (PGIA) is a murine model for rheumatoid arthritis (RA), which is characterized by a Th1 dominance at the onset of the disease. In addition to CD4+ T cells, antigen-presenting B cells and autoantibodies seem to play an important role in the development and regulation of PGIA. To identify proteoglycan-specific CD4+ T cell subsets and Th1- and Th2-supported antibody isotypes during the progression of PGIA, spleen cells of proteoglycan-immunized BALB/c mice were harvested at different times of immunization, and at different stages of the disease, and their cytokine production and antigen-specific antibody isotype profiles were determined by enzyme-linked immunospot (ELISPOT) assays. Both Th1 and Th2 cytokine-producing cells, with the predominance of IL-4/IL-5-secreting cells, were detected during the prearthritic stage, and a shift toward a Th1 dominance was observed at the time of onset of arthritis. Tissue homogenates of acutely inflamed joints contained significantly higher levels of interferon-gamma than IL-4. The prearthritic period and both the acute and chronic phases of joint inflammation were characterized by IgG1 dominance in the sera and this correlated with the number of IgG1-secreting B cells in the spleen. However, the ratio of autoreactive IgG1/IgG2a-secreting cells decreased in arthritic animals. These results indicate the activation and possible regulatory roles of both Th1 and Th2 subsets in the autoimmune process, with the necessity of a relative increase of autoreactive Th1 cells for the induction of joint inflammation.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical And Experimental Immunology. - 120 : 1 (2000), p. 167-173. -
További szerzők:Glant Tibor T. Garzó M. Finnegan, Alison Mikecz Katalin Buzás Edit
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5.

001-es BibID:BIBFORM013556
Első szerző:Kreuz, W.
Cím:A multi-centre study of efficacy and safety of Intratect®, a novel intravenous immunoglobulin preparation / Kreuz W., Erdös M., Rossi P., Bernatowska E., Espanol T., Maródi L.
Dátum:2010
ISSN:0009-9104
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
egyetemen (Magyarországon) készült közlemény
Megjelenés:Clinical And Experimental Immunology. - 161 : 3 (2010), p. 512-517. -
További szerzők:Erdős Melinda (1975-) (infektológus, gyermekimmunológus) Rossi, P. Bernatowska, Ewa Maródi László (1949-) (gyermekgyógyász infektológus, immunológus) Espanol, Teresa
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6.

001-es BibID:BIBFORM040011
035-os BibID:(scopus)0035653055 (wos)000175437200014
Első szerző:Maródi László (gyermekgyógyász infektológus, immunológus)
Cím:Cytokine receptor signalling in neonatal macrophages : defective STAT-1 phosphorylation in response to stimulation with IFN-gamma / Marodi, L., Goda, K., Palicz, A., Szabo, G.
Dátum:2001
ISSN:0009-9104
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Clinical And Experimental Immunology. - 126 : 3 (2001), p. 456-460. -
További szerzők:Goda Katalin (1969-) (biofizikus) Palicz Anita Szabó Gábor (1953-) (biofizikus)
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DOI
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7.

001-es BibID:BIBFORM016247
Első szerző:Maródi László (gyermekgyógyász infektológus, immunológus)
Cím:Down-regulation of Th1 responses in human neonates / L. Maródi
Dátum:2002
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical And Experimental Immunology. - 128 : 1 (2002), p. 1-2. -
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DOI
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8.

001-es BibID:BIBFORM065633
Első szerző:Murad, Yanal M.
Cím:Molecular manipulation with the arthritogenic epitopes of the G1 domain of human cartilage proteoglycan aggrecan / Y. M. Murad, Z. Szabó, K. Ludányi, T. T. Glant
Dátum:2005
ISSN:0009-9104
Megjegyzések:Systemic immunization of BALB/c mice with human cartilage proteoglycan (PG) aggrecan induces progressive polyarthritis. The G1 domain of the PG aggrecan molecule contains most of the T cell epitopes, including three immunodominant ('arthritogenic') and at least six subdominant T cell epitopes. The three dominant T cell epitopes (P49, P70 and P155) were deleted individually or in combination by site directed mutagenesis, and the recombinant human G1 (rhG1) domain (wild type and mutated) proteins were used for immunization. Close to 100% of BALB/c mice immunized with the wild-type (nonmutated) rhG1 domain developed severe arthritis, which was 75% in the absence of P70 (5/4E8) epitope, and very low (< 10% incidence) when all three dominant T cell epitopes were deleted. The onset was delayed and the severity of arthritis reduced in animals when dominant T cell epitopes were missing from the immunizing rhG1 domain. The lack of T cell response to the deleted epitope(s) was specific, but the overall immune response against the wild-type rhG1 domain of human PG was not significantly affected. This study helped us to understand the dynamics and immune-regulatory mechanisms of arthritis, and supported the hypothesis that the development of autoimmune arthritis requires a concerted T cell response to multiple epitopes, rather than the immune response to a single arthritogenic structure.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
aggrecan
animal model
arthritis
rheumatoid arthritis
T cell epitopes
Megjelenés:Clinical And Experimental Immunology 142 : 2 (2005), p. 303-311. -
További szerzők:Szabó Zoltán (1970-) (belgyógyász, reumatológus) Ludányi Katalin (1975-) (immunológus) Glant Tibor (anatómus)
Pályázati támogatás:OMFB-00541/2004
Egyéb
NIH AR40310
Egyéb
NIH AR45652
Egyéb
NIH AR47657
Egyéb
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9.

001-es BibID:BIBFORM025025
Első szerző:Myrsky, Essi
Cím:Coeliac disease-specific autoantibodies targeted against transglutaminase 2 disturb angiogenesis / Myrsky, E., Kaukinen, K., Syrjänen, M., Korponay-Szabó, I. R., Mäki, M., Lindfors, K.
Dátum:2008
ISSN:0009-9104
Megjegyzések:Coeliac disease is characterized by immunoglobulin-A (IgA)-class autoantibodies targeted against transglutaminase 2 (TG2), a multi-functional protein also with a role in angiogenesis. These antibodies are present in patient serum but are also found bound to TG2 below the epithelial basement membrane and around capillaries in the small intestinal mucosa. Based on these facts and the information that the mucosal vasculature of coeliac patients on a gluten-containing diet is disorganized, we studied whether the coeliac disease-specific autoantibodies targeted against TG2 would disturb angiogenesis. The effects of coeliac disease-specific autoantibodies on in vitro angiogenesis were studied in angiogenic cell cultures. The binding of the antibodies to cells, endothelial sprouting, migration of both endothelial and vascular mesenchymal cells, the integrity of the actin cytoskeleton in both cell types and the differentiation of vascular mesenchymal cells were recorded. In vitro, IgA derived from coeliac disease patients on a gluten-containing diet binds to surface TG2 on endothelial and vascular mesenchymal cells and this binding can be inhibited by the removal of TG2. In addition, coeliac disease-specific autoantibodies targeting TG2 disturb several steps of angiogenesis: endothelial sprouting and the migration of both endothelial and vascular mesenchymal cells. Furthermore, the autoantibodies cause disorganization of the actin cytoskeleton in both capillary cell types that account most probably for the defective cellular migration. We conclude that coeliac disease-specific autoantibodies recognizing TG2 inhibit angiogenesis in vitro. This disturbance of the angiogenic process could lead in vivo to the disruption of the mucosal vasculature seen in coeliac disease patients on a gluten-containing diet.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical And Experimental Immunology. - 152 : 1 (2008), p. 111-119. -
További szerzők:Kaukinen, Katri Syrjänen, Mari Korponay-Szabó Ilma (1959-) (gyermekgyógyász) Mäki, Markku Lindfors, Katri
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DOI
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10.

001-es BibID:BIBFORM049586
Első szerző:Szabó Attila (molekuláris biológus, immunológus, filozófus)
Cím:Collaboration of Toll-like and RIG-I-like receptors in human dendritic cells : tRIGgering antiviral innate immune responses / Attila Szabó, Éva Rajnavölgyi
Dátum:2013
ISSN:2164-7712
Megjegyzések:Dendritic cells (DCs) represent a functionally diverse and flexible population of rare cells with the unique capability of binding, internalizing and detecting various microorganisms and their components. However, the response of DCs to innocuous or pathogenic microbes is highly dependent on the type of microbe-associated molecular patterns (MAMPs) recognized by pattern recognition receptors (PRRs) that interact with phylogenetically conserved and functionally indispensable microbial targets that involve both self and foreign structures such as lipids, carbohydrates, proteins, and nucleic acids. Recently, special attention has been drawn to nucleic acid receptors that are able to evoke robust innate immune responses mediated by type I interferons and inflammatory cytokine production against intracellular pathogens. Both conventional and plasmacytoid dendritic cells (cDCs and pDCs) express specific nucleic acid recognizing receptors, such as members of the membrane Toll-like receptor (TLR) and the cytosolic RIG-I-like receptor (RLR) families. TLR3, TLR7/TLR8 and TLR9 are localized in the endosomal membrane and are specialized for the recognition of viral double-stranded RNA, single-stranded RNA, and nonmethylated DNA, respectively whereas RLRs (RIG-I, MDA5, and LGP2) are cytosolic proteins that sense various viral RNA species. In this review we discuss the significance of detecting the genomic content of viruses by DC subsets capable of linking innate and adaptive immunity, and several viral evasion mechanisms that may allow us to better understand these responses. A particular attention is paid to the possible collaboration of TLR and RLR sensors in anti-viral protection.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Pattern recognition receptors
cross-talk
dendritic cell subsets
inflammation
interferon
Megjelenés:American Journal of Clinical and Experimental Immunology. - 2 : 3 (2013), p. 195-207. -
További szerzők:Rajnavölgyi Éva (1950-) (immunológus)
Pályázati támogatás:TÁMOP-4.2.2.A-11/1/KONV-2012-0023
TÁMOP
NK 101538
OTKA
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11.

001-es BibID:BIBFORM061473
Első szerző:Szabó Krisztina (Molekuláris biológus)
Cím:A comprehensive investigation on the distribution of circulating follicular T helper cells and B cell subsets in primary Sjögren's syndrome and systemic lupus erythematosus / Szabó, K., Papp, G., Szántó, A., Tarr, T., Zeher, M.
Dátum:2016
ISSN:0009-9104
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical And Experimental Immunology 183 : 1 (2016), p. 76-89. -
További szerzők:Papp Gábor (1984-) (belgyógyász) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus)
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12.

001-es BibID:BIBFORM032783
035-os BibID:PMID:19664141
Első szerző:Szodoray Péter (belgyógyász, orvos)
Cím:Cells with regulatory function of the innate and adaptive immune system in primary Sjögren's syndrome / Szodoray Péter, Papp Gábor, Horváth Ildikó, Baráth Sándor, Sipka Sándor, Nakken Britt, Zeher Margit
Dátum:2009
Megjegyzések:The aim of the present study was to describe subsets of cells with regulatory properties in primary Sjögren's syndrome (pSS), and to correlate these cell populations with clinical symptoms. Among the 32 investigated patients, 23 had extraglandular manifestations (EGMs), while nine had only glandular symptoms. Twenty healthy individuals served as controls. The percentages of natural killer (NK), natural killer T cells (NK T), interleukin (IL)-10 producing T regulatory type 1 (Tr1) cells and CD4(+)CD25(+) regulatory T cells (T(reg)) cells were determined by flow cytometry and serum cytokine levels of IL-4, IL-6, IL-10, tumour necrosis factor (TNF)-alpha and interferon (IFN)-gamma were evaluated by enzyme-linked immunosorbent assay (ELISA). Functional tests were carried out to assess the suppressor properties of T(reg) cells in patients and controls. Peripheral NK, NK T and Tr1 cell percentages were elevated in pSS, while CD4(+)CD25(+) T(reg) cells showed reduced frequencies in patients compared to controls. In pSS, elevated percentages of NK T, Tr1 and CD4(+)CD25(+) T(reg) cells were observed in patients with EGMs, when compared to patients with sicca symptoms only. CD4(+)CD25(+) T(reg) cell percentages showed a negative correlation with sialometry values. The in vitro functional assay demonstrated lower suppression activity of CD4(+)CD25(+) T(reg) cells in patients compared to controls. Serum IL-6 and TNF-alpha levels were elevated, while IL-10 was decreased in patients compared to controls. Negative correlation was found between IL-10 levels and the percentages of Tr1 cells. Changes in the investigated subsets of regulatory cells in pSS may contribute to the development and progression of the disease.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
egyetemen (Magyarországon) készült közlemény
Megjelenés:Clinical and Experimental Immunology. - 157 : 3 (2009), p. 343-349. -
További szerzők:Papp Gábor (1984-) (belgyógyász) Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Baráth Sándor (1977-) (biológus) Sipka Sándor (1945-) (laboratóriumi szakorvos) Nakken, Britt Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus)
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