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Genetics Home Reference: your guide to understanding genetic conditions
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DICER1

Reviewed May 2013

What is the official name of the DICER1 gene?

The official name of this gene is “dicer 1, ribonuclease type III.”

DICER1 is the gene's official symbol. The DICER1 gene is also known by other names, listed below.

What is the normal function of the DICER1 gene?

The DICER1 gene provides instructions for making a protein that plays a role in regulating the activity (expression) of other genes. The Dicer protein aids in the production of a molecule called microRNA (miRNA). MicroRNAs are short lengths of RNA, a chemical cousin of DNA. Dicer cuts (cleaves) precursor RNA molecules to produce miRNA.

MicroRNAs control gene expression by blocking the process of protein production. In the first step of making a protein from a gene, another type of RNA called messenger RNA (mRNA) is formed and acts as the blueprint for protein production. MicroRNAs attach to specific mRNA molecules and stop the process by which protein is made. Sometimes, miRNAs break down the mRNA, which also blocks protein production. Through this role in regulating the expression of genes, Dicer is involved in many processes, including cell growth and division (proliferation) and the maturation of cells to take on specialized functions (differentiation).

How are changes in the DICER1 gene related to health conditions?

DICER1 syndrome - caused by mutations in the DICER1 gene

Mutations in the DICER1 gene cause DICER1 syndrome. People with this condition have an increased risk of developing many types of tumors, particularly certain tumors of the lungs (pleuropulmonary blastoma); kidneys (cystic nephroma); ovaries (Sertoli-Leydig tumors); and thyroid, a butterfly-shaped gland in the lower neck (multinodular goiter). Most of these mutations lead to an abnormally short Dicer protein that is likely unable to produce miRNA. Without regulation by miRNA, genes are expressed abnormally, which could cause cells to grow and divide uncontrollably and lead to tumor formation.

Where is the DICER1 gene located?

Cytogenetic Location: 14q32.13

Molecular Location on chromosome 14: base pairs 95,086,227 to 95,157,421

The DICER1 gene is located on the long (q) arm of chromosome 14 at position 32.13.

The DICER1 gene is located on the long (q) arm of chromosome 14 at position 32.13.

More precisely, the DICER1 gene is located from base pair 95,086,227 to base pair 95,157,421 on chromosome 14.

See How do geneticists indicate the location of a gene? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genelocation) in the Handbook.

Where can I find additional information about DICER1?

You and your healthcare professional may find the following resources about DICER1 helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the DICER1 gene or gene products?

  • DCR1
  • Dicer
  • Dicer1, Dcr-1 homolog
  • dicer 1, double-stranded RNA-specific endoribonuclease
  • DICER_HUMAN
  • endoribonuclease Dicer
  • helicase MOI
  • helicase-moi
  • helicase with RNAse motif
  • HERNA
  • K12H4.8-LIKE
  • KIAA0928
  • MNG1

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What glossary definitions help with understanding DICER1?

cell ; differentiation ; DNA ; expressed ; gene ; gene expression ; goiter ; helicase ; messenger RNA ; molecule ; motif ; mRNA ; nephroma ; precursor ; proliferation ; protein ; RNA ; syndrome ; thyroid ; tumor

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

References

  • Bahubeshi A, Bal N, Rio Frio T, Hamel N, Pouchet C, Yilmaz A, Bouron-Dal Soglio D, Williams GM, Tischkowitz M, Priest JR, Foulkes WD. Germline DICER1 mutations and familial cystic nephroma. J Med Genet. 2010 Dec;47(12):863-6. doi: 10.1136/jmg.2010.081216. Epub 2010 Oct 29. (http://www.ncbi.nlm.nih.gov/pubmed/21036787?dopt=Abstract)
  • Carthew RW, Sontheimer EJ. Origins and Mechanisms of miRNAs and siRNAs. Cell. 2009 Feb 20;136(4):642-55. doi: 10.1016/j.cell.2009.01.035. Review. (http://www.ncbi.nlm.nih.gov/pubmed/19239886?dopt=Abstract)
  • OMIM: DICER, DROSOPHILA, HOMOLOG OF, 1 (http://omim.org/entry/606241)
  • Ghildiyal M, Zamore PD. Small silencing RNAs: an expanding universe. Nat Rev Genet. 2009 Feb;10(2):94-108. doi: 10.1038/nrg2504. Review. (http://www.ncbi.nlm.nih.gov/pubmed/19148191?dopt=Abstract)
  • NCBI Gene (http://www.ncbi.nlm.nih.gov/gene/23405)
  • Rio Frio T, Bahubeshi A, Kanellopoulou C, Hamel N, Niedziela M, Sabbaghian N, Pouchet C, Gilbert L, O'Brien PK, Serfas K, Broderick P, Houlston RS, Lesueur F, Bonora E, Muljo S, Schimke RN, Bouron-Dal Soglio D, Arseneau J, Schultz KA, Priest JR, Nguyen VH, Harach HR, Livingston DM, Foulkes WD, Tischkowitz M. DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors. JAMA. 2011 Jan 5;305(1):68-77. doi: 10.1001/jama.2010.1910. (http://www.ncbi.nlm.nih.gov/pubmed/21205968?dopt=Abstract)
  • Schultz KA, Pacheco MC, Yang J, Williams GM, Messinger Y, Hill DA, Dehner LP, Priest JR. Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol. 2011 Aug;122(2):246-50. doi: 10.1016/j.ygyno.2011.03.024. Epub 2011 Apr 17. (http://www.ncbi.nlm.nih.gov/pubmed/21501861?dopt=Abstract)
  • Slade I, Bacchelli C, Davies H, Murray A, Abbaszadeh F, Hanks S, Barfoot R, Burke A, Chisholm J, Hewitt M, Jenkinson H, King D, Morland B, Pizer B, Prescott K, Saggar A, Side L, Traunecker H, Vaidya S, Ward P, Futreal PA, Vujanic G, Nicholson AG, Sebire N, Turnbull C, Priest JR, Pritchard-Jones K, Houlston R, Stiller C, Stratton MR, Douglas J, Rahman N. DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet. 2011 Apr;48(4):273-8. doi: 10.1136/jmg.2010.083790. Epub 2011 Jan 25. (http://www.ncbi.nlm.nih.gov/pubmed/21266384?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: May 2013
Published: October 27, 2014