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Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat.
Biochem J. 1994 Jul 01; 301 (Pt 1):1-4.BJ

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.

Authors+Show Affiliations

Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 63110.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

8037655

Citation

Yamaguchi, Y, et al. "Expression of the Wilson Disease Gene Is Deficient in the Long-Evans Cinnamon Rat." The Biochemical Journal, vol. 301 (Pt 1), 1994, pp. 1-4.
Yamaguchi Y, Heiny ME, Shimizu N, et al. Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat. Biochem J. 1994;301 (Pt 1):1-4.
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. The Biochemical Journal, 301 (Pt 1), 1-4.
Yamaguchi Y, et al. Expression of the Wilson Disease Gene Is Deficient in the Long-Evans Cinnamon Rat. Biochem J. 1994 Jul 1;301 (Pt 1):1-4. PubMed PMID: 8037655.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat. AU - Yamaguchi,Y, AU - Heiny,M E, AU - Shimizu,N, AU - Aoki,T, AU - Gitlin,J D, PY - 1994/7/1/pubmed PY - 1994/7/1/medline PY - 1994/7/1/entrez SP - 1 EP - 4 JF - The Biochemical journal JO - Biochem. J. VL - 301 (Pt 1) N2 - 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. SN - 0264-6021 UR - https://www.unboundmedicine.com/medline/citation/8037655/Expression_of_the_Wilson_disease_gene_is_deficient_in_the_Long_Evans_Cinnamon_rat_ L2 - https://portlandpress.com/biochemj/article-lookup/doi/10.1042/bj3010001 DB - PRIME DP - Unbound Medicine ER -