In this study, we conducted a USP cleavage assay and a meta-analysis of the published literature (up to 31 May 2013) to evaluate the impact of five frequent mutations (NM_031907.1: c.363_364insACA, c.494T>C, c.1423C>T, c.1090C>T, c.1737G>A) on enzymatic activity of the USP26 and to assess the strength of the association between those mutations and male infertility.