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Genetics Home Reference: your guide to understanding genetic conditions
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RNASE gene family

Reviewed August 2012

What are the RNASE genes?

Genes in the RNASE gene family provide instructions for making enzymes called ribonucleases (RNases). RNases are enzymes that break down RNA, a chemical cousin of DNA. Degrading RNA serves multiple purposes within cells. Cells must be cleared of RNA that is no longer needed so that cells do not become cluttered with waste. Additionally, breaking down the RNA of foreign invaders, such as viruses, is a critical early immune response in fighting infection and mounting an immune attack.

RNASE genes are active (expressed) in many cells and tissues, including different immune system cells. The variety of tissue activity likely supports the ability of RNase enzymes to help fight infection throughout the body.

The enzymes that are produced from members of the RNASE gene family all share a similar structure and enzyme activity. All the genes in the RNASE gene family are found on chromosome 14. Thirteen genes have been identified in the RNASE gene family, but only eight of the genes provide instructions for making functional enzymes. Most of the genes in this family are designated by the letters RNASE followed by a unique number identifier, starting with the number one. For example, the first identified member of this gene family is the gene RNASE1.

Disease-associated mutations have been found in only one RNASE gene family member, the RNASE5 gene (more commonly called ANG). Mutations in the ANG gene are associated with an increased risk of developing amyotrophic lateral sclerosis (ALS), a condition characterized by progressive movement problems and muscle wasting. Some people with ANG gene mutations who develop ALS also develop a condition called frontotemporal dementia (FTD), which is a progressive brain disorder that affects personality, behavior, and language. It is unknown how ANG gene mutations result in ALS or the combination of ALS and FTD.

Which genes are included in the RNASE gene family?

The HUGO Gene Nomenclature Committee (HGNC) provides a list of genes in the RNASE family (http://www.genenames.org/genefamilies/RNASE).

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

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

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

  • amyotrophic lateral sclerosis

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

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

  • Madame Curie Bioscience: RNA Regulation, Connecting Components of the RNA-Infrastructure (http://www.ncbi.nlm.nih.gov/books/NBK53773/)

What glossary definitions help with understanding the RNASE gene family?

chromosome ; dementia ; DNA ; enzyme ; expressed ; gene ; immune response ; immune system ; infection ; RNA ; sclerosis ; tissue ; wasting

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 RNASE gene family.

  • Sorrentino S. The eight human "canonical" ribonucleases: molecular diversity, catalytic properties, and special biological actions of the enzyme proteins. FEBS Lett. 2010 Jun 3;584(11):2194-200. doi: 10.1016/j.febslet.2010.04.018. Epub 2010 Apr 11. Review. (http://www.ncbi.nlm.nih.gov/pubmed/20388512?dopt=Abstract)
  • Dyer KD, Rosenberg HF. The RNase a superfamily: generation of diversity and innate host defense. Mol Divers. 2006 Nov;10(4):585-97. Review. (http://www.ncbi.nlm.nih.gov/pubmed/16969722?dopt=Abstract)
  • Sorrentino S. Human extracellular ribonucleases: multiplicity, molecular diversity and catalytic properties of the major RNase types. Cell Mol Life Sci. 1998 Aug;54(8):785-94. Review. (http://www.ncbi.nlm.nih.gov/pubmed/9760987?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.

 
Reviewed: August 2012
Published: September 8, 2014