Article

Insulin secretion deficits in a Prader-Willi syndrome β-cell model are associated with a concerted downregulation of multiple endoplasmic reticulum chaperones

2021-12-17

Abstract excerpt

<h4>ABSTRACT</h4> Prader-Willi syndrome (PWS) is a multisystem disorder with neurobehavioral, metabolic, and hormonal phenotypes, caused by loss of expression of a paternally-expressed imprinted gene cluster. Prior evidence from a PWS mouse model identified abnormal pancreatic islet development with retention of aged insulin and deficient insulin secretion. To determine the collective roles of PWS genes in β-cell...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: de5ae8a7-d99a-55a4-abc9-0a9c2eeb9728
DOI: 10.1101/2021.12.16.473032

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Insulin secretion deficits in a Prader-Willi syndrome β-cell model are associated with a concerted downregulation of multiple endoplasmic reticulum chaperonesDOI 10.1101/2021.12.16.473032Read

ER stress and cell fate: FKBP2 regulates proinsulin folding and α- vs. β cell differentiation via NFAT and HDAC9Semantic proximity Read

Increased insulin demand promotes while pioglitazone prevents pancreatic beta cell apoptosis in Wfs1 knockout mice.Semantic proximity Read

ISR inhibition reverses pancreatic β-cell failure in Wolfram syndrome modelsSemantic proximity Read

Insulin mutations impair beta-cell development in a patient-derived iPSC model of neonatal diabetesSemantic proximity Read

β-cell NCK1 is reduced in type 2 diabetes, leading to inefficient β-cell UPR and insulin secretion and revealing sex-specific adaptation during metabolic stressSemantic proximity Read

Progressive endoplasmic reticulum stress over time due to human insulin gene mutation contributes to pancreatic beta cell dysfunction.Semantic proximity Read

IER3IP1 is critical for maintaining glucose homeostasis through regulating the endoplasmic reticulum function and survival of β cellsSemantic proximity Read

Mice conditionally lacking the Wolfram gene in pancreatic islet beta cells exhibit diabetes as a result of enhanced endoplasmic reticulum stress and apoptosis.Semantic proximity Read

Hormonal and Metabolic Defects in a Prader-Willi Syndrome Mouse Model with Neonatal Failure to ThriveSemantic proximity Read

IER3IP1 Mutations Cause Neonatal Diabetes Due to Impaired Proinsulin TraffickingSemantic proximity Read

Functional, transcriptomic, and proteomic profiles of human primary and stem cell-derived beta cells in a state of high insulin production and increased fragilitySemantic proximity Read

Single-Cell Transcriptomics Links Loss of Human Pancreatic β-Cell Identity to ER StressSemantic proximity Read