alexa High Expression Of Atp7b MRNA In The Peripheral Blood Mononuclear Cells Of The Long-Evans Cinnamon Rats: An Animal Model Of Wilsons Disease | OMICS International | Abstract
ISSN: 2161-1041

Hereditary Genetics: Current Research
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Research Article

High Expression Of Atp7b MRNA In The Peripheral Blood Mononuclear Cells Of The Long-Evans Cinnamon Rats: An Animal Model Of Wilsons Disease

Kenji Nakayama1*, Yoshinobu Katoh2, Norikazu Shimizu3, Toyo Okui4, Kozo Matsumoto5, Yukiharu Sawada6, Tsugutoshi Aoki7

1 Department of Cancer Omics Research, World-Leading Drug Discovery Research Center, Kyoto University, Japan

2 Department of Biological Science, Hokkaido Institute of Public Health, Japan

3 Second Department of Pediatrics Toho University School of Medicine, Japan

4 Department of Basic Radiology, School of Veterinary Medicine, Rakuno Gakuen University, Japan

5 Department of Laboratory Animal Science, Kyoto Sangyo University, Japan

6 First Department of Pathology, Sapporo Medical University School of Medicine, Japan

7 Toho University, Ota-ku, Tokyo, Japan.

*Corresponding Author:
Department of Cancer Omics Research
World-Leading Drug Discovery Research Center
Kyoto University, Japan
Tel: +81-75-753-4586
Fax: +81-75-753-9255
E-mail:[email protected]

Received Date: April 10, 2012; Accepted Date: August 10, 2012; Published Date: August 16, 2012

Citation: Nakayama K, Katoh Y, Shimizu N, Okui T, Matsumoto K (2012) High Expression of Atp7b mRNA in the Peripheral Blood Mononuclear Cells of the Long- Evans Cinnamon Rats: an Animal Model of Wilson’s Disease. Hereditary Genet 2: 115. doi: 10.4172/2161-1041.1000115

Copyright: © 2012 Nakayama K, 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.


The Long-Evans Cinnamon (LEC) rat is an animal model of Wilson’s disease. The rat has a mutation in the copper (Cu)-transporting P-type ATPase (Atp7b) gene that is homologous to the human Wilson’s disease gene, ATP7B. The LEC rat shows all of the biochemical features of the disease. In this study, we focused on the expression levels of mutant Atp7b mRNAs in the peripheral blood mononuclear cells (PBMCs) of the LEC rats. Using quantitative real-time polymerase chain reaction (quantitative RT-PCR), we analyzed the expression levels of Atp7b mRNAs in the PBMCs cells of both the LEC rats and Long-Evans Agouti (LEA) rats, the latter being utilized as a control for the LEC rat. At the ages of 5 and 8 weeks, the inductions of Atp7b mRNA were manifested in the PBMCs of both male and female LEC rats, while their levels in the livers were significantly lower than those of the LEA rats. These results suggest the diversity of cell-physiological and endocrinological Cu metabolisms between the PBMCs and the livers of the LEC rats. Our findings indicate the possibility of a novel Cu metabolism in the cardiovascular network that is concerned with Atp7b of the PBMCs.


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