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Open Access 01-11-2013 | Meeting abstract

OR6-005 – Cystine crystals activate inflammasomes

Authors: G Prencipe, I Caiello, S Petrini, L Bracci Laudiero, F Emma, F De Benedetti

Published in: Pediatric Rheumatology | Special Issue 1/2013

Excerpt

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 accumulation in the pathogenesis of clinical features of cystinosis is still unclear, although it is well known that cystine levels are directly proportional to disease severity. …

Title: OR6-005 – Cystine crystals activate inflammasomes
Authors: G Prencipe
I Caiello
S Petrini
L Bracci Laudiero
F Emma
F De Benedetti
Publication date: 01-11-2013
Publisher: BioMed Central
Published in: Pediatric Rheumatology / Issue Special Issue 1/2013
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/1546-0096-11-S1-A100

At a glance: The STEP trials

Springer Medicine

OR6-005 – Cystine crystals activate inflammasomes

Excerpt

At a glance: The STEP trials

Springer Medicine

Excerpt

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