PW03-005 - NLRP3-Q705K monocytes do not produce more IL-1B

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PW03-005 - NLRP3-Q705K monocytes do not produce more IL-1B

Introduction PFAPA is a pediatric auto-inflammatory syndrome of unknown etiology, characterized by recurrent fever, aphthosis, pharyngitis and cervical adenitis. Dysregulated monocyte interleukin-1 beta (IL-1b) secretion is thought to play an important role in fever flares. Recently, it was published that Thp1 cells (a monocytic cell line) transduced with the Q705K variant of NLRP3 increased IL...

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C3a modulates IL-1b secretion in human monocytes by regulating ATP efflux and subsequent NLRP3 inflammasome activation

Inflammatory Diseases, King’s College School of Medicine, London, United Kingdom; Institute for Systemic Inflammation Research, University of Lübeck, Lübeck, Germany; Division of Cellular and Molecular Immunology, Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH; and Centre for Complement and Inflammation Research, Department of Medi...

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P02-025 - Homozygous Q705K sequence variant in NLRP3

Case Report The patient, a 12-year-old boy with healthy parents, experienced his first long febrile episode, associated with abdominal pain, aseptic meningitis, spleenitis and increased inflammatory markers, at the age of 2.5 years. The patient was given corticosteroids, and responded well. After the age of three years, the patient developed recurrent febrile episodes (approximately twice a yea...

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Human keratinocytes produce but do not process pro-interleukin-1 (IL-1) beta. Different strategies of IL-1 production and processing in monocytes and keratinocytes.

Keratinocytes comprise the majority of cells in the epidermis, the interleukin-1 rich layer of tissue contiguous with the outside world. Keratinocytes produce IL-1 alpha and beta mRNA in vitro, but only IL-1 alpha biological activity has been identified in keratinocyte cultures. In contrast, monocytes secrete biological activities attributable to both species of IL-1. Using several monoclonal a...

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PW03-007 - NLRP3 genetic variants in Schnitzler’s syndrome

Introduction Schnitzler’s syndrome (SchS) is an autoinflammatory disorder, characterized by chronic urticaria, fever, gammopathy and bone pain. The etiology is unknown, but interleukin-1 (IL-1) inhibition is very effective, like in the cryopyrin associated periodic syndrome (CAPS), that is caused by activating NLRP3 mutations. Previously, a V198M mutation in NLRP3 was reported in one patient wi...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2013

ISSN: 1546-0096

DOI: 10.1186/1546-0096-11-s1-a231