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Full-Text Articles in Life Sciences

The Peroxisome Proliferator-Activated Receptor Alpha (Ppar ) Regulates Bile Acid Biosynthesis., Mary Hunt, Yi-Zeng Yang, Gosta Eggertsen, Claes Carneheim, Mats Gafvels, Curt Einarsson, Stefan Alexson Sep 2000

The Peroxisome Proliferator-Activated Receptor Alpha (Ppar ) Regulates Bile Acid Biosynthesis., Mary Hunt, Yi-Zeng Yang, Gosta Eggertsen, Claes Carneheim, Mats Gafvels, Curt Einarsson, Stefan Alexson

Articles

Fibrates are a group of hypolipidemic agents which efficiently lower serum triglyceride levels by affecting the expression of many genes involved in lipid metabolism. These effects are exerted via the peroxisome proliferator-activated receptor alpha (PPARa). In addition, fibrates also lower serum cholesterol levels, suggesting a possible link between the PPARa and cholesterol metabolism. Bile acid formation represents an important pathway for elimination of cholesterol, and the sterol 12a-hydroxylase is a branch-point enzyme in the bile acid biosynthetic pathway, which determines the ratio of cholic acid to chenodeoxycholic acid. Treatment of mice for one week with the peroxisome proliferator WY-14,643 or …


Typical Friedreich’S Ataxia Without Gaa Expansions And Gaa Epansions Wthout Typical Friedreich’S Ataxia, Dominick Mccabe, Fergus Ryan, D. Moore, Shirley Mcquaid, M. King, A. Kelly, K. Daly, David Barton, R. Murphy Jan 2000

Typical Friedreich’S Ataxia Without Gaa Expansions And Gaa Epansions Wthout Typical Friedreich’S Ataxia, Dominick Mccabe, Fergus Ryan, D. Moore, Shirley Mcquaid, M. King, A. Kelly, K. Daly, David Barton, R. Murphy

Articles

We clinically assessed and performed polymerase chain reaction analysis for the GAA trinucleotide repeat expansion in 103 patients from 73 families in Ireland, with a prior clinical diagnosis of Friedreich’s ataxia (FA) or an unclassified progressive ataxic syndrome. The patients were classified as “typical” or “atypical” FA according to Harding’s mandatory clinical diagnostic criteria. All patients underwent blood glucose analysis, and electrocardiography and echocardiography was performed in 99 and 101 patients, respectively. Mutation screening for expanded CAG trinucleotide repeats, associated with spinocerebellar ataxia (SCA) 1, 2, 3 and 6 was performed in 86 patients overall, including all GAA negative patients. …