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USP26

Function

Deubiquitinase regulating several biological processes through the deubiquitination of components of these processes (PubMed:20501646, PubMed:28839133). Involved in somatic cell reprogramming through the 'Lys-48'-linked deubiquitination and stabilization of CBX4 and CBX6, two components of the polycomb-repressive complex 1 (PRC1) (PubMed:28839133). Also deubiquitinates and probably stabilizes the androgen receptor (AR), regulating the androgen receptor signaling pathway (PubMed:20501646). May play a role in spermatogenesis (PubMed:34202084).

Involvement in disease

Spermatogenic failure, X-linked, 6

SPGFX6

A male infertility disorder due to asthenoteratozoospermia and characterized by reduced progressive sperm motility and morphologic sperm abnormalities, such as thin heads and short or coiled flagella.

None

The disease may be caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the peptidase C19 family.

Tissue Specificity

Expressed in testis.

Cellular localization

Alternative names

Ubiquitin carboxyl-terminal hydrolase 26, Deubiquitinating enzyme 26, Ubiquitin thioesterase 26, Ubiquitin-specific-processing protease 26, USP26

swissprot:Q9BXU7 entrezGene:83844 omim:300309