Up-regulation of the extracellular matrix remodeling genes, biglycan, neutrophil gelatinase-associated lipocalin, and matrix metalloproteinase-9 in familial amyloid polyneuropathy

SPECIFIC AIMFamilial amyloid polyneuropathy (FAP) is characterized by the extracellular deposition of transthyretin (TTR) aggregates and amyloid fibrils in tissues, particularly in the peripheral nervous system (PNS), and is accompanied by changes in connective tissue. Changes in proteoglycan type and distribution could account at least in part for the altered physical properties observed during disease progression in tissues with TTR deposition, but information is lacking concerning the molecular events that underlie tissue remodeling in FAP. Identification of genes and proteins differentially expressed during pathological changes throughout the course of FAP is needed. FAP salivary glands (SG) were used to identify differentially expressed genes related to tissue remodeling during the course of FAP.PRINCIPAL FINDINGS1. Microarray analysis of FAP salivary glands identified increased expression of genes related to extracellular matrix remodelingTo test whether SG from FAP patients follow signaling pathway...