alexa Mahvash Disease: Pancreatic Neuroendocrine Tumor Syndro
ISSN: 1747-0862

Journal of Molecular and Genetic Medicine
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Review Article

Mahvash Disease: Pancreatic Neuroendocrine Tumor Syndrome Caused by Inactivating Glucagon Receptor Mutation

Matthew B Lucas1, Victoria E Yu1 and Run Yu2*

1Harvard-Westlake School, Los Angeles, California, USA

2Division of Endocrinology, Cedars-Sinai Medical Center, Los Angeles, California, USA

Corresponding Author:
Run Yu
MD, PhD, Division of Endocrinology and Carcinoid and Neuroendocrine Tumor Center
Cedars-Sinai Medical Center, B-1318700
Beverly Blvd, Los Angeles, California 90048, USA
Phone: 310-423-4774
Fax: 310-423-0440

Received Date: September 13, 2013; Accepted Date: October 23, 2013; Published Date: October 28, 2013

Citation: Lucas MB, Yu VE, Yu R (2013) Mahvash Disease: Pancreatic Neuroendocrine Tumor Syndrome Caused by Inactivating Glucagon Receptor Mutation. J Mol Genet Med 7:84. doi:10.4172/1747-0862.1000084

Copyright: © 2013 Lucas MB, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.



Mahvash disease is a novel pancreatic neuroendocrine tumor syndrome caused by inactivating glucagon receptor mutations. Its discovery was triggered by comparison of a patient with pancreatic neuroendocrine tumors, pancreatic α cell hyperplasia, extreme hyperglucagonemia but without glucagonoma syndrome, and occasional hypoglycemia, with the glucagon receptor knockout mice which exhibit similar phenotype and ultimately prove to be a model of Mahvash disease. So far 6 cases have been reported. The inheritance, prevalence, pathogenesis, natural history, diagnosis, treatment, and long-term follow-up of Mahvash disease are discussed in this article. Although rare, Mahvash disease provides important insights into the pathogenesis of pancreatic neuroendocrine tumors, pancreatic α cell fate regulation by glucagon signaling, and safety of glucagon signaling inhibition for diabetes treatment.

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