Conditional deletion of Men1 in the pancreatic β-cell leads to glucagon-expressing tumor development

Feng Li1, Yutong Su, Yulong Cheng

  • 1Department of Endocrinology and Metabolism (F.L., Y.S., Y.Ch., X.J., Y.P., Y.L., J.L., Y.G., Y.Ca., W.W., G.N.), Shanghai Clinical Center for Endocrine and Metabolic Diseases and Shanghai Institute of Endocrinology and Metabolism, Rui-Jin Hospital, Shanghai Jiao-Tong University School of Medicine, and Laboratory of Endocrinology and Metabolism (G.N.), Institute of Health Sciences, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, and Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China; and Laboratoire Génétique Moléculaire, Signalisation et Cancer (C.Z.), Centre National de la Recherche Scientifique, Unité Mixte de Recherche 5201, Faculté de Médecine, Université Claude Bernard Lyon, Centre Leon-Berard, Lyon69366, France.

Endocrinology
|October 25, 2014
PubMed

Insights

Menin (Men1) loss in pancreatic beta cells triggers glucagon-expressing tumor development by altering transcription factors. This study reveals new insights into islet tumorigenesis mechanisms.

Area of Science:

  • Endocrinology
  • Molecular Biology
  • Oncology

Background:

  • The tumor suppressor menin (Men1) is crucial for regulating pancreatic beta-cell proliferation.
  • Understanding the molecular mechanisms driving pancreatic islet tumorigenesis is essential for developing targeted therapies.

Purpose of the Study:

  • To investigate the role of menin in pancreatic beta-cell proliferation and tumorigenesis.
  • To elucidate the molecular changes, including transcription factor alterations, following menin ablation in beta cells.

Main Methods:

  • Utilized a mouse model with floxed Men1 alleles selectively ablated in beta cells using Cre-recombinase under the insulin promoter (RipCre).
  • Analyzed tumor development, including glucagon-expressing tumors and insulinomas, in mutant mice.
  • Assessed the expression of key transcription factors, such as Brn4 and MafB, in tumor cells and examined beta-cell specific transcription factors in mutant beta cells.

Main Results:

  • Menin deficiency in pancreatic beta cells, despite the use of a beta-cell specific Cre-recombinase, led to the development of both glucagon-expressing tumors and insulinomas.
  • The glucagon-expressing tumor cells exhibited menin deficiency and expressed mature alpha-cell transcription factors, Brn4 and MafB.
  • Inactivation of beta-cell specific transcription factors was observed in the menin-deficient beta cells.

Conclusions:

  • Menin ablation in pancreatic beta cells disrupts normal cell function and promotes tumorigenesis.
  • The loss of menin leads to the inactivation of critical transcription factors, driving the development of glucagon-expressing tumors.
  • This research provides novel insights into the pathogenesis of pancreatic islet tumors.

Related Concept Videos

Type I Diabetes II: Pathophysiology01:26

Type I Diabetes II: Pathophysiology

Type 1 diabetes mellitus arises from an immune-mediated destruction of pancreatic β-cells, resulting in an absolute deficiency of insulin. This process develops in genetically susceptible individuals when autoimmunity, environmental exposures, and immunologic dysregulation converge to trigger a targeted attack on the insulin-producing cells of the pancreas. The β-cells are located within the islets of Langerhans and are essential for regulating blood glucose by facilitating cellular...
8
Type I Diabetes I: Introduction01:12

Type I Diabetes I: Introduction

Type 1 diabetes mellitus is a chronic metabolic disorder characterized by an absolute deficiency of insulin resulting from the autoimmune destruction of pancreatic β-cells. Although it can occur at any age, it is most commonly diagnosed in childhood, adolescence, or early adulthood. The loss of insulin production impairs cellular glucose uptake, resulting in persistent hyperglycemia and necessitating lifelong insulin therapy.Autoimmune Destruction of β-CellsThe hallmark of type 1...
8
Glucose Homeostasis: Pancreatic Islets and Insulin Secretion01:27

Glucose Homeostasis: Pancreatic Islets and Insulin Secretion

The pancreatic islets comprising only 1%-2% of the volume are highly vascularized and innervated mini-organs. They contain five endocrine cell types, including β cells that secrete insulin, which is synthesized as a single polypeptide chain, preproinsulin, processed to proinsulin, and finally to insulin and C-peptide. This process is complex and regulated, involving the Golgi complex, the endoplasmic reticulum, and the secretory granules of the β cell.
Insulin and C-peptide are...
2.7K
Insulin Secretory Vesicles01:05

Insulin Secretory Vesicles

Insulin secretory vesicles release insulin to stimulate blood glucose uptake and regulate carbohydrate metabolism. When the blood glucose levels increase, glucose enters the pancreatic β-islet cells through glucose transporters. Once inside, glucose is metabolized through glycolysis, the citric acid cycle, and the electron transport chain, producing ATP. This increase in ATP concentration closes ATP-sensitive potassium channels, leading to depolarization of the membrane and the opening of...
6.2K
Hormones Regulating Blood Glucose01:16

Hormones Regulating Blood Glucose

Insulin is released by beta cells of the pancreas when blood glucose levels are high. It facilitates glucose absorption and utilization in insulin-dependent cells with insulin receptors on their plasma membranes. Insulin promotes glucose uptake by increasing the number of glucose transport proteins in the cell membrane, allowing glucose to enter the cell. As a result, glucose utilization and ATP production are enhanced.
In addition to accelerating glucose uptake and utilization, insulin has...
7.2K