USP26

Last updated
ubiquitin specific peptidase 26
Identifiers
SymbolUSP26
Entrez 83844
HUGO 13485
OMIM 300309
RefSeq NM_031907
UniProt Q9BXU7
Other data
EC number 3.1.2.15
Locus Chr. X q26.2

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]

Deubiquitinating enzyme

Deubiquitinating enzymes (DUBs), also known as deubiquitinating peptidases, deubiquitinating isopeptidases, deubiquitinases, ubiquitin proteases, ubiquitin hydrolases, ubiquitin isopeptidases, are a large group of proteases that cleave ubiquitin from proteins and other molecules. Ubiquitin is attached to proteins in order to regulate the degradation of proteins via the proteasome and lysosome; coordinate the cellular localisation of proteins; activate and inactivate proteins; and modulate protein-protein interactions. DUBs can reverse these effects by cleaving the peptide or isopeptide bond between ubiquitin and its substrate protein. In humans there are nearly 100 DUB genes, which can be classified into two main classes: cysteine proteases and metalloproteases. The cysteine proteases comprise ubiquitin-specific proteases (USPs), ubiquitin C-terminal hydrolases (UCHs), Machado-Josephin domain proteases (MJDs) and ovarian tumour proteases (OTU). The metalloprotease group contains only the Jab1/Mov34/Mpr1 Pad1 N-terminal+ (MPN+) (JAMM) domain proteases.

Contents

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]

Male infertility refers to a male's inability to cause pregnancy in a fertile female. In humans it accounts for 40–50% of infertility. It affects approximately 7% of all men. Male infertility is commonly due to deficiencies in the semen, and semen quality is used as a surrogate measure of male fecundity.

See also

Related Research Articles

USP9Y protein-coding gene in the species Homo sapiens

Ubiquitin specific peptidase 9, Y-linked , also known as USP9Y, is an enzyme which in humans is encoded by the USP9Y gene. It is required for sperm production. This enzyme is a member of the peptidase C19 family and is similar to ubiquitin-specific proteases, which cleave the ubiquitin moiety from ubiquitin-fused precursors and ubiquitinylated proteins.

USP7 protein-coding gene in the species Homo sapiens

Ubiquitin-specific-processing protease 7 (USP7), also known as ubiquitin carboxyl-terminal hydrolase 7 or herpesvirus-associated ubiquitin-specific protease (HAUSP), is an enzyme that in humans is encoded by the USP7 gene.

USP5 protein-coding gene in the species Homo sapiens

Ubiquitin specific peptidase 5 is an enzyme that in humans is encoded by the USP5 gene.

USP9X protein-coding gene in the species Homo sapiens

Probable ubiquitin carboxyl-terminal hydrolase FAF-X is an enzyme that in humans is encoded by the USP9X gene.

USP8 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 8 is an enzyme that in humans is encoded by the USP8 gene.

USP10 protein-coding gene in the species Homo sapiens

Ubiquitin specific peptidase 10, also known as USP10, is an enzyme which in humans is encoded by the USP10 gene.

USP16 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 16 is an enzyme that in humans is encoded by the USP16 gene.

USP4 protein-coding gene in the species Homo sapiens

Ubiquitin specific protease 4 (USP4) is an enzyme that cleaves ubiquitin from a number of protein substrates. Prior to the standardization of nomenclature USP4 was known as UNP, and was one of the first deubiquitinating enzymes to be identified in mammals. In the mouse and human the USP4 protein is encoded by a gene containing 22 exons.

USP11 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase or Ubiquitin specific protease 11 is an enzyme that in humans is encoded by the USP11 gene. USP11 belongs to the Ubiquitin specific proteases family (USPs) which is a sub-family of the Deubiquitinating enzymes (DUBs).USPs are multiple domain proteases and belong to the C19 cysteine proteases sub‒family. Depending on their domain architecture and position there is different homology between the various members. Generally the largest domain is the catalytic domain which harbours the three residue catalytic triad that is included inside conserved motifs. The catalytic domain also contains sequences that are not related with the catalysis function and their role is mostly not clearly understood at present, the length of these sequences varies for each USP and therefore the length of the whole catalytic domain can range from approximately 295 to 850 amino acids. Particular sequences inside the catalytic domain or at the N‒terminus of some USPs have been characterised as UBL and DUSP domains respectively. In some cases, regarding the UBL domains, it has been reported to have a catalysis enhancing function as in the case of USP7. In addition, a so‒called DU domain module is the combination of a DUSP domain followed by a UBL domain separated by a linker and is found in USP11 as well as in USP15 and USP4.

USP36 protein-coding gene in the species Homo sapiens

Ubiquitin-specific protease 36 is an enzyme that in humans is encoded by the USP36 gene.

USP1 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 1 is an enzyme that in humans is encoded by the USP1 gene.

USP15 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 15 is an enzyme that in humans is encoded by the USP15 gene.

USP33 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 33 is an enzyme that in humans is encoded by the USP33 gene.

USP48 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 48 is an enzyme that in humans is encoded by the USP48 gene.

USP2 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 2 is an enzyme that in humans is encoded by the USP2 gene.

USP14 protein-coding gene in the species Homo sapiens

Ubiquitin-specific protease 14 is an enzyme that in humans is encoded by the USP14 gene.

USP20 protein-coding gene in the species Homo sapiens

Ubiquitin carboxyl-terminal hydrolase 20 is an enzyme that in humans is encoded by the USP20 gene.

BPY2 protein-coding gene in the species Homo sapiens

Testis-specific basic protein Y 2 also known as basic charge, Y-linked 2 is a protein that in humans is encoded by the BPY2 gene which resides on the Y chromosome.

USP25 protein-coding gene in the species Homo sapiens

Ubiquitin specific peptidase 25 is a protein that in humans is encoded by the USP25 gene.

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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