The CENPC antibody is an autoantibody targeting centromere protein C (CENP-C), a critical component of the centromere, a chromosomal region essential for proper chromosome segregation during cell division. CENP-C is part of the constitutive centromere-associated network (CCAN) and localizes to the inner kinetochore plate, where it interacts with other centromeric proteins (e.g., CENP-A, CENP-B) and DNA to maintain centromere integrity and mediate microtubule attachment.
CENPC antibodies are primarily associated with autoimmune conditions, particularly systemic sclerosis (scleroderma), where they are classified as a subset of antinuclear antibodies (ANA). They are also detected in other autoimmune diseases, such as systemic lupus erythematosus (SLE) and primary biliary cholangitis (PBC). These antibodies were first identified in the 1980s alongside other anticentromere antibodies (ACA), which typically target CENP-A, CENP-B, and CENP-C.
Clinically, CENPC antibodies serve as diagnostic markers, especially for limited cutaneous systemic sclerosis (lcSSc) or CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia). Their presence correlates with specific disease phenotypes and may predict clinical progression. Detection methods include immunofluorescence assays (IFA) and ELISA. While their exact pathogenic role remains unclear, their formation is linked to genetic susceptibility, epigenetic dysregulation, and environmental triggers. Research continues to explore their mechanistic involvement in autoimmunity and potential as prognostic indicators.