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Article

Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway.

Proceedings of the National Academy of Sciences of the United States of America - 11 Nov 2003

Shoham Nitza G, Centola Michael, Mansfield Elizabeth, Hull Keith M, Wood Geryl, Wise Carol A, Kastner Daniel L

Abstract excerpt

Pyrin, the familial Mediterranean fever protein, is found in association with the cytoskeleton in myeloid/monocytic cells and modulates IL-1beta processing, NF-kappaB activation, and apoptosis. These effects are mediated in part through cognate interactions with the adaptor protein ASC, which shares an N-terminal motif with pyrin. We sought additional upstream regulators of inflammation by using pyrin as the bait...

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Identifiers

PMID: 14595024
DOI: 10.1073/pnas.2135380100
PMCID: PMC263843

Genes

PSTPIP1

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway.PMID 14595024Read ↗

The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1beta production.Semantic proximity Read ↗

Pyrin activates the ASC pyroptosome in response to engagement by autoinflammatory PSTPIP1 mutants.Semantic proximity Read ↗

Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder.Semantic proximity Read ↗

Pyrin, product of the MEFV locus, interacts with the proapoptotic protein, Siva.Semantic proximity Read ↗

A novel Pyrin-Associated Autoinflammation with Neutrophilic Dermatosis mutation further defines 14-3-3 binding of pyrin and distinction to Familial Mediterranean Fever.Semantic proximity Read ↗

Pyrin Modulates the Intracellular Distribution of PSTPIP1.Semantic proximity Read ↗

The immunological function of familial Mediterranean fever disease protein PyrinSemantic proximity Read ↗

The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-kappaB through its N-terminal fragment.Semantic proximity Read ↗

Excess Serum Interleukin-18 Distinguishes Patients with Pathogenic Mutations in <i>PSTPIP1</i>Semantic proximity Read ↗

Familial autoinflammation with neutrophilic dermatosis reveals a regulatory mechanism of pyrin activation.Semantic proximity Read ↗

Inflammation in mice ectopically expressing human Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome-associated PSTPIP1 A230T mutant proteins.Semantic proximity Read ↗

The F-BAR protein PSTPIP1 controls extracellular matrix degradation and filopodia formation in macrophages.Semantic proximity Read ↗

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