To review the current understanding of CENP-A maintenance and function in sperm (Fig. 1), it is important to revisit studies on the autoimmune disorder systemic scleroderma (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia or CREST syndrome). Here, CENPA is linked to systemic sclerosis.