ubiquitin specific peptidase 26
Identifiers
SymbolUSP26
NCBI gene83844
HGNC13485
OMIM300309
RefSeqNM_031907
UniProtQ9BXU7
Other data
EC number3.1.2.15
LocusChr. X q26.2
Search for
StructuresSwiss-model
DomainsInterPro

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]

See also

References

  1. 1 2 Stouffs K, Lissens W, Tournaye H, Van Steirteghem A, Liebaers I (March 2005). "Possible role of USP26 in patients with severely impaired spermatogenesis". European Journal of Human Genetics. 13 (3): 336–40. doi:10.1038/sj.ejhg.5201335. PMID 15562280.
  2. ↑ 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. PMID 29111204.
  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. doi:10.1016/j.bbrc.2016.10.135. ISSN 0006-291X. PMID 27810359.
  4. ↑ Zhang J, Qiu SD, Li SB, Zhou DX, Tian H, Huo YW, Ge L, Zhang QY (November 2007). "Novel mutations in ubiquitin-specific protease 26 gene might cause spermatogenesis impairment and male infertility". Asian Journal of Andrology. 9 (6): 809–14. doi:10.1111/j.1745-7262.2007.00305.x. PMID 17968467.
  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 (October 2006). "Haplotypes, mutations and male fertility: the story of the testis-specific ubiquitin protease USP26". Molecular Human Reproduction. 12 (10): 643–6. doi:10.1093/molehr/gal063. PMID 16888075.


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