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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)
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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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
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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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
Internet cím:	Szerző által megadott URL Intézményi repozitóriumban (DEA) tárolt változat
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