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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Zollinger-Ellison syndrome


Other Names for this Disease
  • Gastrinoma
  • Pancreatic ulcerogenic tumor syndrome
  • Z E syndrome
  • ZES
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Cause


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What causes Zollinger-Ellison syndrome?

In most people with Zollinger-Ellison syndrome (ZES), the cause is not known. However, in about 25-30% of cases, it occurs with an inherited condition called multiple endocrine neoplasia type 1 (MEN1). This condition is caused by changes (mutations) in the MEN1 gene and is inherited in an autosomal dominant manner. Most, but not all, individuals with an MEN1 gene mutation will develop symptoms of multiple endocrine neoplasia type 1.[1][2]

The MEN1 gene normally regulates the body's production of a protein thought to play a role in preventing the development of tumors (a tumor suppressor gene). Mutations that affect the function of this gene therefore affect the body's ability to prevent the growth of tumors, thus leading the the signs and symptoms of multiple endocrine neoplasia type 1.[2]
Last updated: 1/14/2014

References
  1. Zollinger-Ellison Syndrome. National Digestive Diseases Information Clearinghouse (NDDIC). December 24, 2013; http://www.digestive.niddk.nih.gov/ddiseases/pubs/zollinger/. Accessed 1/14/2014.
  2. Zollinger Ellison syndrome. NORD. January 20, 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/360/viewAbstract. Accessed 1/14/2014.