The homeodomain-containing transcription factors Arx and Pax4 control enteroendocrine subtype specification in mice

PLoS One. 2012;7(5):e36449. doi: 10.1371/journal.pone.0036449. Epub 2012 May 3.

Abstract

Intestinal hormones are key regulators of digestion and energy homeostasis secreted by rare enteroendocrine cells. These cells produce over ten different hormones including GLP-1 and GIP peptides known to promote insulin secretion. To date, the molecular mechanisms controlling the specification of the various enteroendocrine subtypes from multipotent Neurog3(+) endocrine progenitor cells, as well as their number, remain largely unknown. In contrast, in the embryonic pancreas, the opposite activities of Arx and Pax4 homeodomain transcription factors promote islet progenitor cells towards the different endocrine cell fates. In this study, we thus investigated the role of Arx and Pax4 in enteroendocrine subtype specification. The small intestine and colon of Arx- and Pax4-deficient mice were analyzed using histological, molecular, and lineage tracing approaches. We show that Arx is expressed in endocrine progenitors (Neurog3(+)) and in early differentiating (ChromograninA(-)) GLP-1-, GIP-, CCK-, Sct- Gastrin- and Ghrelin-producing cells. We noted a dramatic reduction or a complete loss of all these enteroendocrine cell types in Arx mutants. Serotonin- and Somatostatin-secreting cells do not express Arx and, accordingly, the differentiation of Serotonin cells was not affected in Arx mutants. However, the number of Somatostatin-expressing D-cells is increased as Arx-deficient progenitor cells are redirected to the D-cell lineage. In Pax4-deficient mice, the differentiation of Serotonin and Somatostatin cells is impaired, as well as of GIP and Gastrin cells. In contrast, the number of GLP-1 producing L-cells is increased concomitantly with an upregulation of Arx. Thus, while Arx and Pax4 are necessary for the development of L- and D-cells respectively, they conversely restrict D- and L-cells fates suggesting antagonistic functions in D/L cell allocation. In conclusion, these finding demonstrate that, downstream of Neurog3, the specification of a subset of enteroendocrine subtypes relies on both Arx and Pax4, while others depend only on Arx or Pax4.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Basic Helix-Loop-Helix Transcription Factors / genetics
  • Basic Helix-Loop-Helix Transcription Factors / metabolism
  • Cell Differentiation / genetics
  • Cell Lineage / genetics
  • Enteroendocrine Cells / classification
  • Enteroendocrine Cells / cytology
  • Enteroendocrine Cells / metabolism*
  • Gene Expression
  • Gene Expression Regulation
  • Glucagon-Like Peptide 1 / genetics
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / metabolism*
  • Intestinal Mucosa / metabolism
  • Mice
  • Mice, 129 Strain
  • Mice, Knockout
  • Models, Biological
  • Mutation
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism
  • Paired Box Transcription Factors / deficiency
  • Paired Box Transcription Factors / genetics
  • Paired Box Transcription Factors / metabolism*
  • Peptide Hormones / genetics
  • Somatostatin / genetics
  • Transcription Factors / deficiency
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*

Substances

  • ARX protein, mouse
  • Basic Helix-Loop-Helix Transcription Factors
  • Homeodomain Proteins
  • Nerve Tissue Proteins
  • Neurog3 protein, mouse
  • Paired Box Transcription Factors
  • Pax4 protein, mouse
  • Peptide Hormones
  • Transcription Factors
  • Somatostatin
  • Glucagon-Like Peptide 1