Immune mechanisms in spontaneously occurring CIDP in NOD mice

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease affecting the peripheral nervous system (PNS) and is thought to involve both cellular and humoral immunity. Although its etiology remains to be fully elucidated, the use of animal models has provided some important information regarding its pathogenetic mechanisms. The development of a spontaneous autoimmune polyneuropathy (SAP) in B7-2 knockout non-obese diabetic (NOD) mice underscores the importance of co-stimulatory pathways such as B7-1/B7-2:CD28/CTLA-4 molecules in inflammatory neuropathies. These co-stimulatory molecules regulate the balance between pathogenic and regulatory T cells (Tregs). In SAP, pathogenic T cells are directed against myelin protein zero (P0), the most prominent PNS myelin protein that is a member of immunoglobulin gene superfamily.