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Cited by 607

The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.

An Autophagy-Enhancing Drug Promotes Degradation of Mutant α 1 -Antitrypsin Z and Reduces Hepatic Fibrosis

Hidvegi, Tunda, Ewing, Michael, Hale, Pamela, Dippold, Christine, Beckett, Caroline, Kemp, Carolyn, Maurice, Nicholas, Mukherjee, Amitava, Goldbach, Christina, Watkins, Simon, Michalopoulos, George, Perlmutter, David H.·2010·Science

Abstract

Correcting a Liver Problem: The classical form of α 1 -antitrypsin (AT) deficiency is caused by a point mutation that alters the folding and causes intracellular aggregation of AT—an abundant liver-derived plasma glycoprotein. AT deficiency is the most common genetic cause of liver disease in childhood and can also lead to cirrhosis and/or hepatocellular carcinoma in adulthood. Carbamazepine is a drug known to be well tolerated in humans that enhances the intracellular degradation process known as autophagy. Now, Hidvegi et al. (p. 229 , published online June 3; see the Perspective by Sifers ) show that carbamazepine can reduce the severity of liver disease in a mouse model of AT deficiency by enhancing the degradation of misfolded accumulated AT.

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Crossref · 10.1126/science.1190354

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Metadata

Authors
Hidvegi, T., Ewing, M., Hale, P., Dippold, C., Beckett, C., Kemp, C., Maurice, N., Mukherjee, A., Goldbach, C., Watkins, S., Michalopoulos, G., & Perlmutter, D. H.
Venue
Science
Published
2010-07-09
Volume
329
Issue
5988
Pages
229-232
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2094898345
Cited by
607
References
3
FWCI
34.17
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: An Autophagy-Enhancing Drug Promotes Degradation of Mutant α 1 -Antitrypsin Z and Reduces Hepatic Fibrosis. https://app.cochat.ai/papers/10.1126/science.1190354
The paper
Hidvegi, T., Ewing, M., Hale, P., Dippold, C., Beckett, C., Kemp, C., Maurice, N., Mukherjee, A., Goldbach, C., Watkins, S., Michalopoulos, G., & Perlmutter, D. H. (2010). An Autophagy-Enhancing Drug Promotes Degradation of Mutant α 1 -Antitrypsin Z and Reduces Hepatic Fibrosis. Science. https://doi.org/10.1126/science.1190354

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

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.