ReviewCurrent opinion in hematology2010
Neutropenia in type Ib glycogen storage disease.
Review in Current opinion in hematology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 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
39 citing papers in PubMed, 94 citations in OpenAlex.
- Clinical Outcomes and Management in Late Diagnosed Siblings Affected With Attenuated GSD Ib.JIMD reports · 2026Article
- Insights into Neutrophil Dysfunction in Inherited Metabolic Disorders.Journal of innate immunity · 2026Review
- Structures of human glucose-6-phosphate transporter reveal reciprocal antiport mechanism driving glucose-6-phosphate and inorganic phosphate exchange.Nature communications · 2025Article
- Structural basis for transport and inhibition of the human glucose-6-phosphate transporter G6PT.Nature communications · 2025Article
- Gastrointestinal complications of hepatic glycogen storage disease: a national survey questionnaire study in China.Orphanet journal of rare diseases · 2025Article
- Metabolism: a potential regulator of neutrophil fate.Frontiers in immunology · 2024Review
- Glycogen storage diseases: An update.World journal of gastroenterology · 2023Review
- Treatment of the Neutropenia Associated with GSD1b and G6PC3 Deficiency with SGLT2 Inhibitors.Diagnostics (Basel, Switzerland) · 2023Review
- Gene therapy and genome editing for type I glycogen storage diseases.Frontiers in molecular medicine · 2023Review
- Untargeted metabolomic profiling in a patient with glycogen storage disease Ib receiving empagliflozin treatment.JIMD reports · 2022Article
- Molecular mechanisms of aberrant neutrophil differentiation in glycogen storage disease type Ib.Cellular and molecular life sciences : CMLS · 2022Article
- Minimally invasive surgery for glycogen storage disease combined with inflammatory bowel disease: A case report.World journal of clinical cases · 2021Article
- Stem Cell Applications in Lysosomal Storage Disorders: Progress and Ongoing Challenges.Advances in experimental medicine and biology · 2021Article
- A novel SLC37A4 missense mutation in GSD-Ib without hepatomegaly causes enhanced leukocytes endoplasmic reticulum stress and apoptosis.Molecular genetics & genomic medicine · 2021Article
- Improved inflammatory bowel disease, wound healing and normal oxidative burst under treatment with empagliflozin in glycogen storage disease type Ib.Orphanet journal of rare diseases · 2020Article
- Infectious and digestive complications in glycogen storage disease type Ib: Study of a French cohort.Molecular genetics and metabolism reports · 2020Article
- Prevalence and clinical profile of glycogen storage diseases in children from Western India.Clinical and experimental hepatology · 2020Article
- Mutation analysis ofThe Journal of international medical research · 2019Article
- Hepatomegaly with neutropenia: a girl with glycogen storage disease Ib.BMJ case reports · 2019Article
- Neutropenia in the age of genetic testing: Advances and challenges.American journal of hematology · 2019Review
Corrections and comments
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Authors and funding
3 authors at 1 institution in 3 countries.
Funding
Abstract
purpose of reviewGlycogen storage disease type Ib, characterized by disturbed glucose homeostasis, neutropenia, and neutrophil dysfunction, is caused by a deficiency in a ubiquitously expressed glucose-6-phosphate transporter (G6PT). G6PT translocates glucose-6-phosphate (G6P) from the cytoplasm into the lumen of the endoplasmic reticulum, in which it is hydrolyzed to glucose either by a liver/kidney/intestine-restricted glucose-6-phosphatase-alpha (G6Pase-alpha) or by a ubiquitously expressed G6Pase-beta. The role of the G6PT/G6Pase-alpha complex is well established and readily explains why G6PT disruptions disturb interprandial blood glucose homeostasis. However, the basis for neutropenia and neutrophil dysfunction in glycogen storage disease type Ib is poorly understood. Recent studies that are now starting to unveil the mechanisms are presented in this review. RECENT
findingsCharacterization of G6Pase-beta and generation of mice lacking either G6PT or G6Pase-beta have shown that neutrophils express the G6PT/G6Pase-beta complex capable of producing endogenous glucose. Loss of G6PT activity leads to enhanced endoplasmic reticulum stress, oxidative stress, and apoptosis that underlie neutropenia and neutrophil dysfunction in glycogen storage disease type Ib. SUMMARY: Neutrophil function is intimately linked to the regulation of glucose and G6P metabolism by the G6PT/G6Pase-beta complex. Understanding the molecular mechanisms that govern energy homeostasis in neutrophils has revealed a previously unrecognized pathway of intracellular G6P metabolism in neutrophils.
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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.