USP26 explained

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,^[4] specifically Sertoli cell-only syndrome, and an absence of sperm in the ejaculate (azoospermia).^[5]

See also

• Male infertility

Notes and References

1. Stouffs K, Lissens W, Tournaye H, Van Steirteghem A, Liebaers I . Possible role of USP26 in patients with severely impaired spermatogenesis . European Journal of Human Genetics . 13 . 3 . 336–40 . March 2005 . 15562280 . 10.1038/sj.ejhg.5201335 . free .
2. Liu YL, Zheng J, Mi YJ, Zhao J, Tian QB . The impacts of nineteen mutations on the enzymatic activity of USP26 . Gene . 641 . 292–296 . January 2018 . 29111204 . 10.1016/j.gene.2017.10.074 .
3. 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. 10.1016/j.bbrc.2016.10.135. 27810359. 0006-291X.
4. Zhang J, Qiu SD, Li SB, Zhou DX, Tian H, Huo YW, Ge L, Zhang QY . Novel mutations in ubiquitin-specific protease 26 gene might cause spermatogenesis impairment and male infertility . Asian Journal of Andrology . 9 . 6 . 809–14 . November 2007 . 17968467 . 10.1111/j.1745-7262.2007.00305.x . free .
5. Ravel C, El Houate B, Chantot S, Lourenço D, Dumaine A, Rouba H, Bandyopadahyay A, Radhakrishna U, Das B, Sengupta S, Mandelbaum J, Siffroi JP, McElreavey K . Haplotypes, mutations and male fertility: the story of the testis-specific ubiquitin protease USP26 . Molecular Human Reproduction . 12 . 10 . 643–6 . October 2006 . 16888075 . 10.1093/molehr/gal063 . free .