USP26

{{Short description|Mammalian protein found in Homo sapiens}}

{{protein

|Name=ubiquitin specific peptidase 26

|caption=

|image=

|width=

|HGNCid=13485

|Symbol=USP26

|AltSymbols=

|EntrezGene=83844

|OMIM=300309

|RefSeq=NM_031907

|UniProt=Q9BXU7

|PDB=

|ECnumber=3.1.2.15

|Chromosome=X

|Arm=q

|Band=26.2

|LocusSupplementaryData=

}}

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.{{cite journal | vauthors = Stouffs K, Lissens W, Tournaye H, Van Steirteghem A, Liebaers I | title = Possible role of USP26 in patients with severely impaired spermatogenesis | journal = European Journal of Human Genetics | volume = 13 | issue = 3 | pages = 336–40 | date = March 2005 | pmid = 15562280 | doi = 10.1038/sj.ejhg.5201335 | doi-access = free }} 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.{{cite journal | vauthors = Liu YL, Zheng J, Mi YJ, Zhao J, Tian QB | title = The impacts of nineteen mutations on the enzymatic activity of USP26 | journal = Gene | volume = 641 | pages = 292–296 | date = January 2018 | pmid = 29111204 | doi = 10.1016/j.gene.2017.10.074 }} 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.{{Cite journal|last=Lahav-Baratz|first=Shirly|last2=Kravtsova-Ivantsiv|first2=Yelena|last3=Golan|first3=Shay|last4=Ciechanover|first4=Aaron|date=January 2017|title=The testis-specific USP26 is a deubiquitinating enzyme of the ubiquitin ligase Mdm2|journal=Biochemical and Biophysical Research Communications|volume=482|issue=1|pages=106–111|doi=10.1016/j.bbrc.2016.10.135|pmid=27810359|issn=0006-291X}}

Recent research has suggested that defects in USP26 may be involved in some cases of male infertility,{{cite journal | vauthors = Zhang J, Qiu SD, Li SB, Zhou DX, Tian H, Huo YW, Ge L, Zhang QY | title = Novel mutations in ubiquitin-specific protease 26 gene might cause spermatogenesis impairment and male infertility | journal = Asian Journal of Andrology | volume = 9 | issue = 6 | pages = 809–14 | date = November 2007 | pmid = 17968467 | doi = 10.1111/j.1745-7262.2007.00305.x | doi-access = free }} specifically Sertoli cell-only syndrome, and an absence of sperm in the ejaculate (azoospermia).{{cite journal | vauthors = 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 | title = Haplotypes, mutations and male fertility: the story of the testis-specific ubiquitin protease USP26 | journal = Molecular Human Reproduction | volume = 12 | issue = 10 | pages = 643–6 | date = October 2006 | pmid = 16888075 | doi = 10.1093/molehr/gal063 | doi-access = free }}