Ubiquitin carboxyl-terminal hydrolase 26 is an enzyme that in humans is encoded by the USP26gene. It functions as a peptidase that specifically targets ubiquitin.
The USP26 gene is an X-linked gene exclusively expressed in the testis and it codes for the ubiquitin-specific protease 26. 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. 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.
Recent research has suggested that defects in USP26 may be involved in some cases of male infertility, specifically Sertoli cell-only syndrome, and an absence of sperm in the ejaculate (azoospermia).
↑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 S, Kravtsova-Ivantsiv Y, Golan S, Ciechanover A (January 2017). "The testis-specific USP26 is a deubiquitinating enzyme of the ubiquitin ligase Mdm2". Biochemical and Biophysical Research Communications. 482 (1): 106–111. Bibcode:2017BBRC..482..106L. doi:10.1016/j.bbrc.2016.10.135. PMID27810359.
.svg){kind=small}