POGZ and Intellectual disability: Although the biological mechanism that links CHAMP1 mutations and intellectual disability is unknown, the pathogenic mechanism appears to be a result of the loss of the C-terminal region of CHAMP1. POGZ binds to the C-terminal region of CHAMP1 and is critical for proper chromosome segregation.3,11 Therefore, loss of the C-terminus may impair CHAMP1 chromosomal localization or/and its binding to other proteins, such as POGZ.