DOI: 10.1042/bj3010001. Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat

Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat

10.1042/bj3010001

Crossref journal-article

Portland Press Ltd.

Biochemical Journal (288)

Abstract

Long-Evans Cinnamon rats develop a necrotizing hepatitis characterized by excessive hepatic copper accumulation, defective holoceruloplasmin biosynthesis and impaired biliary copper excretion. To elucidate the molecular basis of this defect, a cDNA clone encoding the rat Wilson disease gene was isolated and used to examine gene expression in selected tissues from normal and Long-Evans Cinnamon rats. Although this cDNA readily detects Wilson transcripts in liver and other tissues from normal rats, such transcripts are entirely absent from tissues derived from the Long-Evans Cinnamon rat strain. These data therefore identify the Long-Evans Cinnamon rat as the first bona fide animal model of Wilson disease and suggest that this rat strain may be a valuable resource in the study of this genetic disorder.

Bibliography

Yamaguchi, Y., Heiny, M. E., Shimizu, N., Aoki, T., & Gitlin, J. D. (1994). Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat. Biochemical Journal, 301(1), 1â4.

Authors 5

Y Yamaguchi (first)
M E Heiny (additional)
N Shimizu (additional)
T Aoki (additional)
J D Gitlin (additional)

References 0 Referenced 67

None

Dates

Type	When
Created	10 years ago (Aug. 10, 2015, 5:38 p.m.)
Deposited	3 years, 9 months ago (Nov. 23, 2021, 5:57 p.m.)
Indexed	2 months, 2 weeks ago (June 10, 2025, 1:28 p.m.)
Issued	31 years, 1 month ago (July 1, 1994)
Published	31 years, 1 month ago (July 1, 1994)
Published Print	31 years, 1 month ago (July 1, 1994)

Funders 0

None

BibTeX

@article{Yamaguchi_1994, title={Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat}, volume={301}, ISSN={1470-8728}, url={http://dx.doi.org/10.1042/bj3010001}, DOI={10.1042/bj3010001}, number={1}, journal={Biochemical Journal}, publisher={Portland Press Ltd.}, author={Yamaguchi, Y and Heiny, M E and Shimizu, N and Aoki, T and Gitlin, J D}, year={1994}, month=jul, pages={1–4} }

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