A Biochemical Abnormality in Cerebrotendinous Xanthomatosis IMPAIRMENT OF BILE ACID BIOSYNTHESIS ASSOCIATED WITH INCOMPLETE DEGRADATION OF THE CHOLESTEROL SIDE CHAIN

Por Setoguchi, T., Salen, Gerald, Tint, G. S., Mosbach, E. H.
Publicado em 1974

Dietary xenosterols lead to infertility and loss of abdominal adipose tissue in sterolin-deficient mice

Por Solca, Curzio, Tint, G. Stephen, Patel, Shailendra B.
Publicado em 2013

Increased formation of ursodeoxycholic acid in patients treated with chenodeoxycholic acid.

Por Salen, G, Tint, G S, Eliav, B, Deering, N, Mosbach, E H
Publicado em 1974

The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols

Por Tint, G. S., Yu, Hongwei, Shang, Quan, Xu, Guorong, Patel, Shailendra B.
Publicado em 2006

Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26.

Por Salen, G, Shefer, S, Tint, G S, Nicolau, G, Dayal, B, Batta, A K
Publicado em 1985

Loss of apolipoprotein E exacerbates the neonatal lethality of the Smith-Lemli-Opitz syndrome mouse

Por Solcà, Curzio, Pandit, Bhaswati, Yu, Hongwei, Tint, G. Stephen, Patel, Shailendra B.
Publicado em 2007

Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats.

Por Xu, G, Salen, G, Shefer, S, Ness, G C, Chen, T S, Zhao, Z, Tint, G S
Publicado em 1995

Abnormal high density lipoproteins in cerebrotendinous xanthomatosis.

Por Shore, V, Salen, G, Cheng, F W, Forte, T, Shefer, S, Tint, G S, Lindgren, F T
Publicado em 1981

Biosynthesis of Chenodeoxycholic Acid in Man: STEREOSPECIFIC SIDE-CHAIN HYDROXYLATIONS OF 5β-CHOLESTANE-3α, 7α-DIOL

Por Shefer, S., Cheng, F. W., Batta, A. K., Dayal, B., Tint, G. S., Salen, G.
Publicado em 1978

Cholic Acid Biosynthesis: THE ENZYMATIC DEFECT IN CEREBROTENDINOUS XANTHOMATOSIS

Por Salen, Gerald, Shefer, S., Cheng, F. W., Dayal, B., Batta, A. K., Tint, G. S.
Publicado em 1979

Competitive inhibition of bile acid synthesis by endogenous cholestanol and sitosterol in sitosterolemia with xanthomatosis. Effect on cholesterol 7 alpha-hydroxylase.

Por Shefer, S, Salen, G, Nguyen, L, Batta, A K, Packin, V, Tint, G S, Hauser, S
Publicado em 1988

Normal Cognition and Behavior in a Smith-Lemli-Opitz Syndrome Patient Who Presented With Hirschsprung Disease

Por Mueller, C., Patel, S., Irons, M., Antshel, K., Salen, G., Tint, G.S., Bay, C.
Publicado em 2003

A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.

Por Shefer, S, Cheng, F W, Dayal, B, Hauser, S, Tint, G S, Salen, G, Mosbach, E H
Publicado em 1976

Selective reconstitution of liver cholesterol biosynthesis promotes lung maturation but does not prevent neonatal lethality in Dhcr7 null mice

Por Yu, Hongwei, Li, Man, Tint, G Stephen, Chen, Jianliang, Xu, Guorong, Patel, Shailendra B
Publicado em 2007

Quantifying raft proteins in neonatal mouse brain by 'tube-gel' protein digestion label-free shotgun proteomics

Por Yu, Hongwei, Wakim, Bassam, Li, Man, Halligan, Brian, Tint, G Stephen, Patel, Shailendra B
Publicado em 2007

A molecular defect in hepatic cholesterol biosynthesis in sitosterolemia with xanthomatosis.

Por Nguyen, L B, Shefer, S, Salen, G, Ness, G C, Tint, G S, Zaki, F G, Rani, I
Publicado em 1990

Increasing dietary cholesterol induces different regulation of classic and alternative bile acid synthesis

Por Xu, Guorong, Salen, Gerald, Shefer, Sarah, Tint, G. Stephen, Nguyen, Lien B., Chen, Thomas S., Greenblatt, David
Publicado em 1999

Desmosterol in brain is elevated because DHCR24 needs REST for robust expression but REST is poorly expressed

Por Tint, G.S., Pan, Luxing, Shang, Quan, Sharpe, Laura J., Brown, Andrew J., Li, Man, Yu, Hongwei
Publicado em 2014

Feedback regulation of bile-acid synthesis in the rat. Differing effects of taurocholate and tauroursocholate.

Por Shefer, S, Nguyen, L, Salen, G, Batta, A K, Brooker, D, Zaki, F G, Rani, I, Tint, G S
Publicado em 1990

Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome

Por Yu, Hongwei, Wessels, Andy, Chen, Jianliang, Phelps, Aimee L, Oatis, John, Tint, G Stephen, Patel, Shailendra B
Publicado em 2004