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Genetics Home Reference: your guide to understanding genetic conditions
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ZC2HC gene family

Reviewed August 2013

What are the ZC2HC genes?

Genes in the ZC2HC family provide instructions for making proteins known as C2HC-type zinc finger proteins. This type is a subset of a much larger group of zinc finger proteins, which are involved in many cellular functions.

Zinc finger proteins contain one or more short regions called zinc finger domains. These regions include a specific pattern of protein building blocks (amino acids) and one or more charged atoms of zinc (zinc ions). C2HC refers to the particular sequence of the amino acids cysteine and histidine found in C2HC-type zinc finger proteins. These amino acids fold around one or more zinc ions, making the protein very stable and enabling it to attach (bind) firmly to other molecules.

Little is known about the function of C2HC-containing zinc finger proteins. Unlike many other zinc finger proteins, most proteins of this type do not appear to bind to DNA. However, these proteins may interact with RNA, a chemical cousin of DNA. C2HC-containing zinc finger proteins may also help to modify other proteins.

Which genes are included in the ZC2HC gene family?

The HUGO Gene Nomenclature Committee (HGNC) provides a list of genes in the ZC2HC family (http://www.genenames.org/genefamilies/ZC2HC).

Genetics Home Reference summarizes the normal function and health implications of these members of the ZC2HC gene family: IKBKG and KAT6B.

What conditions are related to genes in the ZC2HC gene family?

Genetics Home Reference includes these conditions related to genes in the ZC2HC gene family:

  • anhidrotic ectodermal dysplasia with immune deficiency
  • genitopatellar syndrome
  • incontinentia pigmenti
  • Ohdo syndrome, Say-Barber-Biesecker-Young-Simpson variant
  • osteopetrosis

Where can I find additional information about the ZC2HC gene family?

You may find the following resources about the ZC2HC gene family helpful.

  • RCSC Protein Data Bank: Zinc Fingers (http://www.rcsb.org/pdb/101/motm.do?momID=87)
  • Molecular Cell Biology (fourth edition, 2000): Zinc Finger Proteins (http://www.ncbi.nlm.nih.gov/books/NBK21572/)
  • InterPro: Zinc Finger, C2HC-Type (http://www.ebi.ac.uk/interpro/entry/IPR002515)

What glossary definitions help with understanding the ZC2HC gene family?

acids ; B-cells ; cysteine ; DNA ; enhancer ; gene ; histidine ; ions ; kinase ; lysine ; protein ; RNA

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 ZC2HC gene family.

  • Akhtar A, Becker PB. The histone H4 acetyltransferase MOF uses a C2HC zinc finger for substrate recognition. EMBO Rep. 2001 Feb;2(2):113-8. (http://www.ncbi.nlm.nih.gov/pubmed/11258702?dopt=Abstract)
  • Notenboom V, Hibbert RG, van Rossum-Fikkert SE, Olsen JV, Mann M, Sixma TK. Functional characterization of Rad18 domains for Rad6, ubiquitin, DNA binding and PCNA modification. Nucleic Acids Res. 2007;35(17):5819-30. Epub 2007 Aug 24. (http://www.ncbi.nlm.nih.gov/pubmed/17720710?dopt=Abstract)
  • Jandrig B, Seitz S, Hinzmann B, Arnold W, Micheel B, Koelble K, Siebert R, Schwartz A, Ruecker K, Schlag PM, Scherneck S, Rosenthal A. ST18 is a breast cancer tumor suppressor gene at human chromosome 8q11.2. Oncogene. 2004 Dec 9;23(57):9295-302. (http://www.ncbi.nlm.nih.gov/pubmed/15489893?dopt=Abstract)
  • Shifera AS. The zinc finger domain of IKKγ (NEMO) protein in health and disease. J Cell Mol Med. 2010 Oct;14(10):2404-14. doi: 10.1111/j.1582-4934.2010.01054.x. Review. (http://www.ncbi.nlm.nih.gov/pubmed/20345847?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 2013
Published: July 21, 2014