Evidence map›Paper›PMID 41101292›Full record

ArticleMolecular genetics and metabolism2025

Novel murine model provides insights into early-onset of kidney disease in glycogen storage disease type Ib.

Kunal Pratap, Cheol Lee, Lisa Zhang, Hung Dar Chen, Irina Arnaoutova, Brian C Mansfield, Janice Y Chou

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors.

Kunal PratapSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Cheol LeeSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Lisa ZhangSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Hung Dar ChenSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Irina ArnaoutovaSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Brian C MansfieldSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Janice Y ChouSection on Cellular Differentiation, Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA. Electronic address: chouja@mail.nih.gov.

Funding

Molecular Genetics Of Heritable Human DisordersZIAHD000912 · NICHD · EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT · PI CHOU, JANICE · 2009 to 2025
$25.1M
MOLECULAR GENETICS OF HERITABLE HUMAN DISORDERSZ01HD000912 · NICHD · EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT · PI CHOU, JANICE · 1985 to 2008
$3.5M
Intramural NIH HHS Z01 HD000912Intramural NIH HHS ZIA HD000912
6 · The paper itself

Abstract

Glycogen storage disease type Ib (GSD-Ib), caused by a deficiency of the glucose-6-phosphate transporter (G6PT), is characterized by impaired glucose homeostasis and progressive renal dysfunction. Using G6pt-deficient (G6pt-/-) mice, which closely recapitulate the pathophysiology of GSD-Ib, we previously demonstrated that GSD-Ib nephropathy is marked by disrupted renal homeostasis, acute kidney injury, and fibrosis. However, due to the severity of the metabolic defect, G6pt-/- mice typically fail to survive beyond three weeks of age, limiting the study of disease progression in adulthood. To overcome this limitation, we generated liver human G6PT-augmented, kidney G6PT-deficient (L-hG6PT/K

Indexed as

Glycogen Storage Disease Type IKidney DiseasesMonosaccharide Transport ProteinsAnimalsAntiportersDisease Models, AnimalGlucose Transport Proteins, FacilitativeHumansKidneyLiverMiceMice, KnockoutAntiportersglucose 6-phosphate(transporter)Glucose Transport Proteins, FacilitativeMonosaccharide Transport ProteinsKidney-gene knockoutLiver gene augmentationMouse modelWnt/β-catenin signaling

Identifiers

PMID41101292
PMCPMC12539553

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Registered trials

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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.