Journal article

Pyrin Modulates the Intracellular Distribution of PSTPIP1

Andrea L Waite, Philip Schaner, Neil Richards, Banu Balci-Peynircioglu, Seth L Masters, Susannah D Brydges, Michelle Fox, Arthur Hong, Engin Yilmaz, Daniel L Kastner, Ellis L Reinherz, Deborah L Gumucio

PLOS ONE | PUBLIC LIBRARY SCIENCE | Published : 2009

Abstract

PSTPIP1 is a cytoskeleton-associated adaptor protein that links PEST-type phosphatases to their substrates. Mutations in PSTPIP1 cause PAPA syndrome (Pyogenic sterile Arthritis, Pyoderma gangrenosum, and Acne), an autoinflammatory disease. PSTPIP1 binds to pyrin and mutations in pyrin result in familial Mediterranean fever (FMF), a related autoinflammatory disorder. Since disease-associated mutations in PSTPIP1 enhance pyrin binding, PAPA syndrome and FMF are thought to share a common pathoetiology. The studies outlined here describe several new aspects of PSTPIP1 and pyrin biology. We document that PSTPIP1, which has homology to membrane-deforming BAR proteins, forms homodimers and generate..

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University of Melbourne Researchers

Grants

Awarded by NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES


Awarded by NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES


Awarded by NHLBI NIH HHS


Awarded by NIAID NIH HHS