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USP26

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Mammalian protein found in Homo sapiens
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. 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).

See also

References

  1. ^ 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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