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

• Published in: PLoS genetics Vol. 19; no. 4; p. e1010710
• Main Authors: Koppes, Erik A, Johnson, Marie A, Moresco, James J, Luppi, Patrizia, Lewis, Dale W, Stolz, Donna B, Diedrich, Jolene K, Yates, John R, Wek, Ronald C, Watkins, Simon C, Gollin, Susanne M, Park, Hyun Jung, Drain, Peter, Nicholls, Robert D
• Format: Journal Article
• Language: English
• Published: Public Library of Science 17.04.2023
• Subjects: Analysis; Cells; Complications and side effects; Endoplasmic reticulum; Gene expression; Genes; Genetic aspects; Genomes; Genomics; Health aspects; Insulin; Medical research; Medicine, Experimental; Metabolic diseases; Molecular chaperones; Pancreatic beta cells; Physiological aspects; Prader-Willi syndrome; Risk factors; United States

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...