USP26 is a peptidase enzyme. The USP26 gene is an X-linked gene exclusively expressed in the testis and it codes for the ubiquitin-specific protease 26.[1] The USP26 gene is found at Xq26.2 on the X-chromosome as a single exon. The enzyme that this gene encodes comprises 913 amino acid residues and it is 104 kilodalton in size, which is transcribed from a sequence of 2794 nucleotide base-pairs on the X-chromosome.[2] The USP26 enzyme is a deubiquitinating enzyme that places a very significant role in the regulation of protein turnover during spermatogenesis. It is a testis-specific enzyme that is solely express in spermatogonia and can prevent the degradation of ubiquitinated USP26 substrates.[3]
Recent research has suggested that defects in USP26 may be involved in some cases of male infertility,[1][4] specifically Sertoli cell-only syndrome, and an absence of sperm in the ejaculate (azoospermia).[5]
↑Liu YL, Zheng J, Mi YJ, Zhao J, Tian QB (January 2018). "The impacts of nineteen mutations on the enzymatic activity of USP26". Gene. 641: 292–296. doi:10.1016/j.gene.2017.10.074. PMID29111204.
↑Lahav-Baratz, Shirly; Kravtsova-Ivantsiv, Yelena; Golan, Shay; Ciechanover, Aaron (January 2017). "The testis-specific USP26 is a deubiquitinating enzyme of the ubiquitin ligase Mdm2". Biochemical and Biophysical Research Communications. 482 (1): 106–111. doi:10.1016/j.bbrc.2016.10.135. ISSN0006-291X. PMID27810359.