RET and neuroendocrine tumors

Pituitary. 2006;9(3):179-92. doi: 10.1007/s11102-006-0263-4.

Abstract

The RET proto-oncogene encodes a receptor tyrosine kinase that is a main component of the signaling pathway activated by the glial cell line-derived neurotrophic factor family ligands. Gene targeting studies revealed that signaling through RET plays a crucial role in neuronal and renal organogenesis. It is well-known that germline mutations in RET lead to the human inherited diseases, multiple endocrine neoplasia type 2 (MEN 2) and Hirschsprung's disease, and that somatic rearrangements of RET cause papillary thyroid carcinoma. Due to marked advances in understanding of the molecular mechanisms of the development of MEN 2, a consensus on MEN 2 management associated with RET status is being reached and currently put into general use as a guideline. In this review, we summarize progress in the study of RET from bench to bedside, focusing on pathophysiology of neuroendocrine tumors.

Publication types

  • Review

MeSH terms

  • Adrenal Gland Neoplasms / enzymology
  • Animals
  • Biomarkers, Tumor / chemistry
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism*
  • Carcinoma, Medullary / enzymology
  • Carcinoma, Papillary / enzymology
  • Gene Expression Regulation, Enzymologic
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Multiple Endocrine Neoplasia Type 2a / enzymology
  • Multiple Endocrine Neoplasia Type 2b / enzymology
  • Mutation
  • Neuroendocrine Tumors / enzymology*
  • Neuroendocrine Tumors / genetics
  • Neuroendocrine Tumors / pathology
  • Neuroendocrine Tumors / therapy
  • Pheochromocytoma / enzymology
  • Prognosis
  • Protein Conformation
  • Proto-Oncogene Proteins c-ret / chemistry
  • Proto-Oncogene Proteins c-ret / genetics
  • Proto-Oncogene Proteins c-ret / metabolism*
  • Signal Transduction* / genetics
  • Thyroid Neoplasms / enzymology

Substances

  • Biomarkers, Tumor
  • Proto-Oncogene Proteins c-ret
  • RET protein, human