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Cited by 161

The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.

Absence of the SRC-2 Coactivator Results in a Glycogenopathy Resembling Von Gierke's Disease

Open access
Chopra, Atul R., Louet, Jean-Francois, Saha, Pradip, An, Jie, DeMayo, Franco, Xu, Jianming, York, Brian, Karpen, Saul, Finegold, Milton, Moore, David, Chan, Lawrence, Newgard, Christopher B., O'Malley, Bert W.·2008·Science

Abstract

Hepatic glucose production is critical for basal brain function and survival when dietary glucose is unavailable. Glucose-6-phosphatase (G6Pase) is an essential, rate-limiting enzyme that serves as a terminal gatekeeper for hepatic glucose release into the plasma. Mutations in G6Pase result in Von Gierke's disease (glycogen storage disease–1a), a potentially fatal genetic disorder. We have identified the transcriptional coactivator SRC-2 as a regulator of fasting hepatic glucose release, a function that SRC-2 performs by controlling the expression of hepatic G6Pase . SRC-2 modulates G6Pase expression directly by acting as a coactivator with the orphan nuclear receptor RORα. In addition, SRC-2 ablation, in both a whole-body and liver-specific manner, resulted in a Von Gierke's disease phenotype in mice. Our results position SRC-2 as a critical regulator of mammalian glucose production.

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Metadata

Authors
Chopra, A. R., Louet, J.-F., Saha, P., An, J., DeMayo, F., Xu, J., York, B., Karpen, S., Finegold, M., Moore, D., Chan, L., Newgard, C. B., & O'Malley, B. W.
Venue
Science
Published
2008-11-28
Volume
322
Issue
5906
Pages
1395-1399
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2089735989
Cited by
161
References
1
FWCI
10.68
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Absence of the SRC-2 Coactivator Results in a Glycogenopathy Resembling Von Gierke's Disease. https://app.cochat.ai/papers/10.1126/science.1164847
The paper
Chopra, A. R., Louet, J.-F., Saha, P., An, J., DeMayo, F., Xu, J., York, B., Karpen, S., Finegold, M., Moore, D., Chan, L., Newgard, C. B., & O'Malley, B. W. (2008). Absence of the SRC-2 Coactivator Results in a Glycogenopathy Resembling Von Gierke's Disease. Science. https://doi.org/10.1126/science.1164847

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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