Role of tissue inhibitor of metalloproteinases-1 in the development of autoimmune lymphoproliferation.

ABSTRACT:

Background

Inherited defects decreasing function of the Fas death receptor cause autoimmune lymphoproliferative syndrome and its variant Dianzani's autoimmune lymphoproliferative disease. Analysis of the lymphocyte transcriptome from a patient with this latter condition detected striking over-expression of osteopontin and tissue inhibitor of metalloproteinases-1. Since previous work on osteopontin had detected increased serum levels in these patients, associated with variations of its gene, the aim of this work was to extend the analysis to tissue inhibitor of metalloproteinases-1.

Design and methods

Tissue inhibitor of metalloproteinases-1 levels were evaluated in sera and culture supernatants from patients and controls by enzyme-linked immunosorbent assay. Activation- and

SUBMITTER: Boggio E 

PROVIDER: S-EPMC2966912 | biostudies-literature | 2010 Nov

REPOSITORIES: biostudies-literature