Coiled-coil glutamate-rich protein 1 (CCER1)

Coiled-coil glutamate-rich protein 1 (CCER1) is an intrinsically disordered, testis-specific nuclear protein found in vertebrates, highly enriched in round and elongating spermatids during spermiogenesis. CCER1 regulates the histone-to-protamine transition, promoting chromatin compaction required for sperm maturation; it exerts these effects by mediating histone epigenetic modifications, self-assembling into liquid–liquid phase-separated nuclear condensates, and acting as a scaffold for chromatin and histone-modifying enzymes. Loss-of-function mutations or deletions of CCER1 in humans and mice result in defective sperm nuclear condensation and infertility, and rare pathogenic human CCER1 variants are linked to nonobstructive azoospermia. CCER1 is not a classical therapeutic target, with no established roles as an enzyme, receptor, transporter, or signaling molecule, but instead serves as a crucial germline determinant of sperm development and a marker for certain steps of spermatid differentiation.

Other names

Coiled-coil domain-containing glutamate-rich protein 1CCER1C12orf12MGC26598

Not applicable (no drugs targeting CCER1 characterized)