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DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors.

Publication ,  Journal Article
Jiao, Y; Shi, C; Edil, BH; de Wilde, RF; Klimstra, DS; Maitra, A; Schulick, RD; Tang, LH; Wolfgang, CL; Choti, MA; Velculescu, VE; Diaz, LA ...
Published in: Science
March 4, 2011

Pancreatic neuroendocrine tumors (PanNETs) are a rare but clinically important form of pancreatic neoplasia. To explore the genetic basis of PanNETs, we determined the exomic sequences of 10 nonfamilial PanNETs and then screened the most commonly mutated genes in 58 additional PanNETs. The most frequently mutated genes specify proteins implicated in chromatin remodeling: 44% of the tumors had somatic inactivating mutations in MEN1, which encodes menin, a component of a histone methyltransferase complex, and 43% had mutations in genes encoding either of the two subunits of a transcription/chromatin remodeling complex consisting of DAXX (death-domain-associated protein) and ATRX (α thalassemia/mental retardation syndrome X-linked). Clinically, mutations in the MEN1 and DAXX/ATRX genes were associated with better prognosis. We also found mutations in genes in the mTOR (mammalian target of rapamycin) pathway in 14% of the tumors, a finding that could potentially be used to stratify patients for treatment with mTOR inhibitors.

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Published In

Science

DOI

EISSN

1095-9203

Publication Date

March 4, 2011

Volume

331

Issue

6021

Start / End Page

1199 / 1203

Location

United States

Related Subject Headings

  • X-linked Nuclear Protein
  • Tumor Suppressor Proteins
  • Tuberous Sclerosis Complex 2 Protein
  • TOR Serine-Threonine Kinases
  • Signal Transduction
  • Sequence Analysis, DNA
  • Proto-Oncogene Proteins
  • Prognosis
  • Phosphatidylinositol 3-Kinases
  • Pancreatic Neoplasms
 

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Jiao, Y., Shi, C., Edil, B. H., de Wilde, R. F., Klimstra, D. S., Maitra, A., … Papadopoulos, N. (2011). DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science, 331(6021), 1199–1203. https://doi.org/10.1126/science.1200609
Jiao, Yuchen, Chanjuan Shi, Barish H. Edil, Roeland F. de Wilde, David S. Klimstra, Anirban Maitra, Richard D. Schulick, et al. “DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors.Science 331, no. 6021 (March 4, 2011): 1199–1203. https://doi.org/10.1126/science.1200609.
Jiao Y, Shi C, Edil BH, de Wilde RF, Klimstra DS, Maitra A, et al. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011 Mar 4;331(6021):1199–203.
Jiao, Yuchen, et al. “DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors.Science, vol. 331, no. 6021, Mar. 2011, pp. 1199–203. Pubmed, doi:10.1126/science.1200609.
Jiao Y, Shi C, Edil BH, de Wilde RF, Klimstra DS, Maitra A, Schulick RD, Tang LH, Wolfgang CL, Choti MA, Velculescu VE, Diaz LA, Vogelstein B, Kinzler KW, Hruban RH, Papadopoulos N. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011 Mar 4;331(6021):1199–1203.
Journal cover image

Published In

Science

DOI

EISSN

1095-9203

Publication Date

March 4, 2011

Volume

331

Issue

6021

Start / End Page

1199 / 1203

Location

United States

Related Subject Headings

  • X-linked Nuclear Protein
  • Tumor Suppressor Proteins
  • Tuberous Sclerosis Complex 2 Protein
  • TOR Serine-Threonine Kinases
  • Signal Transduction
  • Sequence Analysis, DNA
  • Proto-Oncogene Proteins
  • Prognosis
  • Phosphatidylinositol 3-Kinases
  • Pancreatic Neoplasms