ArticleEndocrinology, diabetes & metabolism case reports2016
Pancreatic α-cell hyperplasia and hyperglucagonemia due to a glucagon receptor splice mutation.
Article in Endocrinology, diabetes & metabolism case reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
31 citing papers in PubMed, 47 citations in OpenAlex.
- Effect of a glucagon receptor antibody (REMD-477) in type 1 diabetes: A randomized controlled trial.Diabetes, obesity & metabolism · 2018Trial
- SEL1L-HRD1 ER-associated degradation facilitates prohormone convertase 2 maturation and glucagon production in islet α cells.Nature communications · 2026Article
- Hepatic CBP/p300 Orchestrate Amino Acid-Driven Gluconeogenesis through Histone Crotonylation.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- The neglected PCK1/glucagon (inter)action in nutrient homeostasis beyond gluconeogenesis: Disease pathogenesis and treatment.Molecular metabolism · 2025Review
- Pancreatic endocrine and exocrine signaling and crosstalk in physiological and pathological status.Signal transduction and targeted therapy · 2025Review
- REVISITING GLUCAGON ACTION IN DIABETES: IS IT ALL BAD?Transactions of the American Clinical and Climatological Association · 2025Review
- Article
- Glucagon infusion alters the circulating metabolome and urine amino acid excretion in dogs.The Journal of endocrinology · 2024Article
- The past, present, and future physiology and pharmacology of glucagon.Cell metabolism · 2022Review
- The Liver-α-Cell Axis in Health and in Disease.Diabetes · 2022Review
- Alpha-cell paracrine signaling in the regulation of beta-cell insulin secretion.Frontiers in endocrinology · 2022Review
- Newly discovered endocrine functions of the liver.World journal of hepatology · 2021Review
- The liver-alpha-cell axis after a mixed meal and during weight loss in type 2 diabetes.Endocrine connections · 2021Article
- Article
- Proglucagon-Derived Peptides as Therapeutics.Frontiers in endocrinology · 2021Review
- Glucagon acutely regulates hepatic amino acid catabolism and the effect may be disturbed by steatosis.Molecular metabolism · 2020Article
- Elevated Serum Amino Acids Induce a Subpopulation of Alpha Cells to Initiate Pancreatic Neuroendocrine Tumor Formation.Cell reports. Medicine · 2020Article
- Global Transcriptomic Analysis of Zebrafish Glucagon Receptor Mutant Reveals Its Regulated Metabolic Network.International journal of molecular sciences · 2020Article
- Integrated Metabolomics and Lipidomics Analysis Reveal Remodeling of Lipid Metabolism and Amino Acid Metabolism in Glucagon Receptor-Deficient Zebrafish.Frontiers in cell and developmental biology · 2020Article
- Glucagon Receptor Signaling and Glucagon Resistance.International journal of molecular sciences · 2019Review
Corrections and comments
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Authors and funding
11 authors at 6 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Glucagon stimulates hepatic glucose production by activating specific glucagon receptors in the liver, which in turn increase hepatic glycogenolysis as well as gluconeogenesis and ureagenesis from amino acids. Conversely, glucagon secretion is regulated by concentrations of glucose and amino acids. Disruption of glucagon signaling in rodents results in grossly elevated circulating glucagon levels but no hypoglycemia. Here, we describe a patient carrying a homozygous G to A substitution in the invariant AG dinucleotide found in a 3' mRNA splice junction of the glucagon receptor gene. Loss of the splice site acceptor consensus sequence results in the deletion of 70 nucleotides encoded by exon 9, which introduces a frame shift and an early termination signal in the receptor mRNA sequence. The mutated receptor neither bound LEARNING POINTS: Loss of function of the glucagon receptor may not necessarily lead to the dysregulation of glucose homeostasis.Loss of function of the glucagon receptor causes hyperaminoacidemia, hyperglucagonemia and α-cell hyperplasia and sometimes other pancreatic abnormalities.A hepato-pancreatic feedback regulation of the α-cells, possibly involving amino acids, may exist in humans.
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.