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A mouse model of sitosterolemia: absence of Abcg8/sterolin-2 results in failure to secrete biliary cholesterol

Creator: Klett, Eric L, Lu, Kangmo, Kosters, Astrid, Vink, Edwin, Lee, Mi-Hye, Altenburg, Michael, Shefer, Sarah, Batta, Ashok K, Yu, Hongwei, Chen, Jianliang, Klein, Richard, Looije, Norbert, Oude-Elferink, Ronald, Groen, Albert K, Maeda, Nobuyo, Salen, Gerald, Patel, Shailendra B

Abstract Background Mutations in either of two genes comprising the STSL locus, ATP-binding cassette (ABC)-transporters ABCG5 (encoding sterolin-1) and ABCG8 (encoding sterolin-2), result in sitosterolemia, a rare autosomal recessive disorder of (more)

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12916_2003_article_8.pdf

Collection: BioMed Central

Date Deposited: 2016-09-23

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Figure 1 Generation of mice deficient in Abcg8/sterolin-2

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 3 Lipid profiles of the plasma of Abcg8+/+, Abcg8+/- and Abcg8-/- mice

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 4 Analyses of mRNA expression and enzyme activity in mouse livers.

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 5 N-glycosylation of Abcg5/sterolin-1 analyses in Abcg8-/- mouse liver

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 7 Immunohistochemical evaluation of mouse Abcg5/sterolin-1 expression in liver

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 8 Biliary sterol, phospholipid and bile salt analyses

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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Figure 9 Biliary cholesterol and sitosterol secretion

Collection: BioMed Central

Date Deposited: 2016-09-23

Date Created: 2016-09-23

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