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

Reviewed November 2008

What are the ZFYVE genes?

Genes in the ZFYVE family provide instructions for making proteins that contain a short region called an FYVE zinc finger. This region includes a specific pattern of protein building blocks (amino acids) and two charged atoms of zinc (zinc ions). FYVE zinc fingers direct the proteins that contain them to various compartments within the cell by attaching (binding) to a substance on the surface of these compartments called phosphatidylinositol-3-phosphate (PI3P).

The FYVE zinc finger was named after the initials of the first four proteins that were found to contain this region: Fab1, YOTB, Vac1, and EEA1. There are now at least 60 proteins that are known to contain an FYVE zinc finger.

Which genes are included in the ZFYVE gene family?

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

Genetics Home Reference summarizes the normal function and health implications of these members of the ZFYVE gene family: FGD1, FGD4, and ZFYVE26.

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

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

  • Aarskog-Scott syndrome
  • Charcot-Marie-Tooth disease
  • spastic paraplegia type 15

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

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

  • National Center For Biotechnology Information (NCBI) Conserved Domains: FYVE (http://www.ncbi.nlm.nih.gov/Structure/cdd/cddsrv.cgi?uid=28947)

What glossary definitions help with understanding the ZFYVE gene family?

acids ; cell ; domain ; ions ; pH ; phosphate ; protein

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 ZFYVE gene family.

  • Wellcome Trust Institute: FYVE Zinc Finger (http://pfam.sanger.ac.uk/family?entry=PF01363)
  • Annual Reviews Collection (2002): FYVE Domains (http://www.ncbi.nlm.nih.gov/books/NBK2234/) (U.S. National Library of Medicine)
  • Stenmark H, Aasland R, Toh BH, D'Arrigo A. Endosomal localization of the autoantigen EEA1 is mediated by a zinc-binding FYVE finger. J Biol Chem. 1996 Sep 27;271(39):24048-54. (http://www.ncbi.nlm.nih.gov/pubmed/8798641?dopt=Abstract)
  • Sankaran VG, Klein DE, Sachdeva MM, Lemmon MA. High-affinity binding of a FYVE domain to phosphatidylinositol 3-phosphate requires intact phospholipid but not FYVE domain oligomerization. Biochemistry. 2001 Jul 24;40(29):8581-7. (http://www.ncbi.nlm.nih.gov/pubmed/11456498?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: November 2008
Published: September 15, 2014