NDLI: Development of hepatocellular adenomas and carcinomas in mice with liver-specific G6Pase-α deficiency

Content Provider	PubMed Central
Author	Roberta, Resaz Vanni, Cristina Daniela, Segalerba Sementa, Angela R. Mastracci, Luca Grillo, Federica Murgia, Daniele Bosco, Maria Carla Chou, Janice Y. Barbieri, Ottavia Luigi, Varesio Eva, Alessandra
Copyright Year	2014
Abstract	Glycogen storage disease type 1a (GSD-1a) is caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α), and is characterized by impaired glucose homeostasis and a high risk of developing hepatocellular adenomas (HCAs). A globally G6Pase-α-deficient (G6pc −/−) mouse model that shows pathological features similar to those of humans with GSD-1a has been developed. These mice show a very severe phenotype of disturbed glucose homeostasis and rarely live beyond weaning. We generated liver-specific G6Pase-α-deficient (LS‑G6pc −/−) mice as an alternative animal model for studying the long-term pathophysiology of the liver and the potential treatment strategies, such as cell therapy. LS‑G6pc −/− mice were viable and exhibited normal glucose profiles in the fed state, but showed significantly lower blood glucose levels than their control littermates after 6 hours of fasting. LS‑G6pc −/− mice developed hepatomegaly with glycogen accumulation and hepatic steatosis, and progressive hepatic degeneration. Ninety percent of the mice analyzed developed amyloidosis by 12 months of age. Finally, 25% of the mice sacrificed at age 10–20 months showed the presence of multiple HCAs and in one case late development of hepatocellular carcinoma (HCC). In conclusion, LS‑G6pc −/− mice manifest hepatic symptoms similar to those of human GSD-1a and, therefore, represent a valid model to evaluate long-term liver pathogenesis of GSD-1a.
Related Links	http://dx.doi.org/10.1242/dmm.014878
Ending Page	1091
Page Count	9
Starting Page	1083
File Format	PDF
ISSN	17548403
e-ISSN	17548411
Journal	Disease Models & Mechanisms
Issue Number	9
Volume Number	7
Language	English
Publisher	The Company of Biologists Limited
Publisher Date	2014-09-01
Access Restriction	Open
Rights Holder	The Company of Biologists Limited
Subject Keyword	Immunology and Microbiology (miscellaneous) Biochemistry, Genetics and Molecular Biology(all) Medicine (miscellaneous) Neuroscience (miscellaneous) Medicine(all) Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Neuroscience Immunology and Microbiology Medicine Biochemistry, Genetics and Molecular Biology

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in

Cardiac fibrosis in mice expressing an inducible myocardial-specific Cre driver

Deficiency of the bone mineralization inhibitor NPP1 protects mice against obesity and diabetes

A high level of liver-specific expression of oncogenic KrasV12 drives robust liver tumorigenesis in transgenic zebrafish

Functional validation of GWAS gene candidates for abnormal liver function during zebrafish liver development

Optineurin deficiency in mice contributes to impaired cytokine secretion and neutrophil recruitment in bacteria-driven colitis

The first transgenic mice: an interview with Mario Capecchi

Ovarian senescence increases liver fibrosis in humans and zebrafish with steatosis

Of Mice and Men, and Medicine: an interview with Monica Justice

Leiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophy

Development of hepatocellular adenomas and carcinomas in mice with liver-specific G6Pase-α deficiency

Similar Documents

Cardiac fibrosis in mice expressing an inducible myocardial-specific Cre driver

Deficiency of the bone mineralization inhibitor NPP1 protects mice against obesity and diabetes

A high level of liver-specific expression of oncogenic KrasV12 drives robust liver tumorigenesis in transgenic zebrafish

Functional validation of GWAS gene candidates for abnormal liver function during zebrafish liver development

Optineurin deficiency in mice contributes to impaired cytokine secretion and neutrophil recruitment in bacteria-driven colitis

The first transgenic mice: an interview with Mario Capecchi

Ovarian senescence increases liver fibrosis in humans and zebrafish with steatosis

Of Mice and Men, and Medicine: an interview with Monica Justice

Leiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophy

Development of hepatocellular adenomas and carcinomas in mice with liver-specific G6Pase-α deficiency