MPZ抗体

Myelin protein zero (MPZ), also known as P0. is a key structural component of myelin in the peripheral nervous system. Encoded by the MPZ gene, this transmembrane glycoprotein constitutes approximately 50% of peripheral nerve myelin, playing a critical role in maintaining myelin compaction through homophilic adhesion. Its immunoglobulin-like extracellular domain mediates membrane adhesion, essential for efficient nerve conduction. Autoantibodies targeting MPZ are associated with autoimmune neuropathies, particularly chronic inflammatory demyelinating polyneuropathy (CIDP) and variants of Guillain-Barré syndrome (GBS). These antibodies may disrupt myelin integrity, leading to demyelination, axonal damage, and clinical manifestations like muscle weakness and sensory deficits. While MPZ antibodies are detected in a subset of patients, their pathogenic role remains debated due to variable clinical correlations. Some studies suggest antibody-mediated complement activation contributes to nerve injury. Diagnostic testing often involves ELISA or cell-based assays, though interpretation requires caution as MPZ antibodies occasionally occur in non-inflammatory conditions or asymptomatic individuals. Research continues to clarify their diagnostic specificity, pathogenic mechanisms, and potential as therapeutic targets in immune-mediated neuropathies.