OR6-005 – Cystine crystals activate inflammasomes

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OR6-005 – Cystine crystals activate inflammasomes

Introduction Nephropathic cystinosis is a rare autosomal recessive disorder caused by a mutation in the CTNS gene, which encodes for cystinosin. It is characterized by the lysosomal accumulation of cystine, which leads to the formation of cystine crystals within various organs, including kidneys, brain, cornea, intestine and bone marrow. The exact role of intralysosomal cystine crystals accumul...

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Intralysosomal cystine crystals in cystinosis.

Electron microscopic study of conjunctival biopsies from members of one sibship each of nephropathic and benign cystinosis showed polymorphic cystine profiles abundantly distributed in fibroblasts and histiocytes. These crystalline spaces measured from 0.2(i to 2/i and were delineated marginally by intact cellular membranes. Small crystalline aggregates were found in acid-phosphatase-positive o...

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Outer Membrane Vesicles Prime and Activate Macrophage Inflammasomes and Cytokine Secretion In Vitro and In Vivo

Outer membrane vesicles (OMVs) are proteoliposomes blebbed from the surface of Gram-negative bacteria. Chronic periodontitis is associated with an increase in subgingival plaque of Gram-negative bacteria, Porphyromonas gingivalis, Treponema denticola, and Tannerella forsythia. In this study, we investigated the immune-modulatory effects of P. gingivalis, T. denticola, and T. forsythia OMVs on m...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2013

ISSN: 1546-0096

DOI: 10.1186/1546-0096-11-s1-a100