USP gene family

Reviewed January 2009

What are the USP genes?

Genes in the USP gene family provide instructions for making more than 60 proteins called ubiquitin-specific proteases. A protease is an enzyme that cuts apart other proteins.

Ubiquitin-specific proteases help to regulate many cellular processes by controlling the amount of the protein ubiquitin. Ubiquitin is an essential part of a multi-step process called the ubiquitin-proteasome system, which helps control the levels of many proteins in the cell. Attaching ubiquitin to another protein serves to target that protein for destruction by the proteasome.

Mutations that affect the function of ubiquitin-specific proteases are associated with a number of cancers as well as other disorders involving inflammation and the regulation of cell death.

Which genes are included in the USP gene family?

The HUGO Gene Nomenclature Committee (HGNC) provides a list of genes in the USP family (http://www.genenames.org/genefamily/usp.php).

Genetics Home Reference summarizes the normal function and health implications of this member of the USP gene family: USP9Y.

What conditions are related to genes in the USP gene family?

Genetics Home Reference includes these conditions related to genes in the USP gene family:

Y chromosome infertility

Where can I find additional information about the USP gene family?

You may find the following resources about the USP gene family helpful.

Eurekah BioScience Collection: Protein Degradation by the UPP (http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=eurekah&part=A65132)

What glossary definitions help with understanding the USP gene family?

cell ; enzyme ; gene ; inflammation ; protease ; proteasome ; protein ; ubiquitin

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://www.ghr.nlm.nih.gov/glossary).

References

These sources were used to develop the Genetics Home Reference summary for the USP gene family.

Daviet L, Colland F. Targeting ubiquitin specific proteases for drug discovery. Biochimie. 2008 Feb;90(2):270-83. Epub 2007 Sep 22. Review. (http://www.ncbi.nlm.nih.gov/pubmed/17961905?dopt=Abstract)
Zhu X, Ménard R, Sulea T. High incidence of ubiquitin-like domains in human ubiquitin-specific proteases. Proteins. 2007 Oct 1;69(1):1-7. (http://www.ncbi.nlm.nih.gov/pubmed/17597129?dopt=Abstract)
Bonnet J, Romier C, Tora L, Devys D. Zinc-finger UBPs: regulators of deubiquitylation. Trends Biochem Sci. 2008 Aug;33(8):369-75. doi: 10.1016/j.tibs.2008.05.005. Epub 2008 Jul 4. (http://www.ncbi.nlm.nih.gov/pubmed/18603431?dopt=Abstract)

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.



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