PubMed

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

Yu H, Wakim B, Li M, Halligan B, Tint GS, Patel SB.

Proteome Sci. 2007 Sep 24;5:17.PMID: 17892558 [PubMed - in process]Related articlesFree article

An overlapping binding site in the CYP7A1 promoter allows activation of FXR to override the stimulation by LXRalpha.

Shang Q, Pan L, Saumoy M, Chiang JY, Tint GS, Salen G, Xu G.

Am J Physiol Gastrointest Liver Physiol. 2007 Oct;293(4):G817-23. Epub 2007 Aug 9.PMID: 17690173 [PubMed - indexed for MEDLINE]Related articlesFree article

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

Yu H, Li M, Tint GS, Chen J, Xu G, Patel SB.

BMC Dev Biol. 2007 Apr 4;7:27.PMID: 17408495 [PubMed - indexed for MEDLINE]Related articlesFree article

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

Solcà C, Pandit B, Yu H, Tint GS, Patel SB.

Mol Genet Metab. 2007 May;91(1):7-14. Epub 2007 Jan 2.PMID: 17197219 [PubMed - indexed for MEDLINE]Related articlesFree article

NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols.

Liou HL, Dixit SS, Xu S, Tint GS, Stock AM, Lobel P.

J Biol Chem. 2006 Dec 1;281(48):36710-23. Epub 2006 Oct 2.PMID: 17018531 [PubMed - indexed for MEDLINE]Related articlesFree article

Cerebrotendinous xanthomatosis: a rare cause of bilateral Achilles tendon swelling and ataxia. A case report.

Brodsky JW, Beischer AD, Anat D, East C, Soltero E, Tint GS, Salen G, Silverman J.

J Bone Joint Surg Am. 2006 Jun;88(6):1340-4. No abstract available. PMID: 16757769 [PubMed - indexed for MEDLINE]Related articles

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

Tint GS, Yu H, Shang Q, Xu G, Patel SB.

J Lipid Res. 2006 Jul;47(7):1535-41. Epub 2006 May 1.PMID: 16651660 [PubMed - indexed for MEDLINE]Related articlesFree article

The stimulatory effect of LXRalpha is blocked by SHP despite the presence of a LXRalpha binding site in the rabbit CYP7A1 promoter.

Shang Q, Pan L, Saumoy M, Chiang JY, Tint GS, Salen G, Xu G.

J Lipid Res. 2006 May;47(5):997-1004. Epub 2006 Feb 17.PMID: 16489206 [PubMed - indexed for MEDLINE]Related articlesFree article

A membrane defect in the pathogenesis of the Smith-Lemli-Opitz syndrome.

Tulenko TN, Boeze-Battaglia K, Mason RP, Tint GS, Steiner RD, Connor WE, Labelle EF.

J Lipid Res. 2006 Jan;47(1):134-43. Epub 2005 Oct 28.PMID: 16258167 [PubMed - indexed for MEDLINE]Related articlesFree article

Partial rescue of neonatal lethality of Dhcr7 null mice by a nestin promoter-driven DHCR7 transgene expression.

Yu H, Wessels A, Tint GS, Patel SB.

Brain Res Dev Brain Res. 2005 Apr 21;156(1):46-60.PMID: 15862627 [PubMed - indexed for MEDLINE]Related articles

FXR-activating ligands inhibit rabbit ASBT expression via FXR-SHP-FTF cascade.

Li H, Chen F, Shang Q, Pan L, Shneider BL, Chiang JY, Forman BM, Ananthanarayanan M, Tint GS, Salen G, Xu G.

Am J Physiol Gastrointest Liver Physiol. 2005 Jan;288(1):G60-6.PMID: 15591588 [PubMed - indexed for MEDLINE]Related articlesFree article

Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis.

Honda A, Salen G, Matsuzaki Y, Batta AK, Xu G, Hirayama T, Tint GS, Doy M, Shefer S.

J Lipid Res. 2005 Feb;46(2):287-96. Epub 2004 Dec 1.PMID: 15576845 [PubMed - indexed for MEDLINE]Related articlesFree article

Long-term effects of sterol depletion in C. elegans: sterol content of synchronized wild-type and mutant populations.

Merris M, Kraeft J, Tint GS, Lenard J.

J Lipid Res. 2004 Nov;45(11):2044-51. Epub 2004 Aug 16.PMID: 15314097 [PubMed - indexed for MEDLINE]Related articlesFree article

Inhibition of ileal bile acid transport lowers plasma cholesterol levels by inactivating hepatic farnesoid X receptor and stimulating cholesterol 7 alpha-hydroxylase.

Li H, Xu G, Shang Q, Pan L, Shefer S, Batta AK, Bollineni J, Tint GS, Keller BT, Salen G.

Metabolism. 2004 Jul;53(7):927-32.PMID: 15254889 [PubMed - indexed for MEDLINE]Related articles

Hydrophilic 7 beta-hydroxy bile acids, lovastatin, and cholestyramine are ineffective in the treatment of cerebrotendinous xanthomatosis.

Batta AK, Salen G, Tint GS.

Metabolism. 2004 May;53(5):556-62.PMID: 15131757 [PubMed - indexed for MEDLINE]Related articles

Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.

Sleat DE, Wiseman JA, El-Banna M, Price SM, Verot L, Shen MM, Tint GS, Vanier MT, Walkley SU, Lobel P.

Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5886-91. Epub 2004 Apr 7.PMID: 15071184 [PubMed - indexed for MEDLINE]Related articlesFree article

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

Yu H, Wessels A, Chen J, Phelps AL, Oatis J, Tint GS, Patel SB.

BMC Dev Biol. 2004 Feb 2;4:1.PMID: 15005800 [PubMed - indexed for MEDLINE]Related articlesFree article

Dietary cholesterol stimulates CYP7A1 in rats because farnesoid X receptor is not activated.

Xu G, Pan LX, Li H, Shang Q, Honda A, Shefer S, Bollineni J, Matsuzaki Y, Tint GS, Salen G.

Am J Physiol Gastrointest Liver Physiol. 2004 May;286(5):G730-5. Epub 2003 Dec 18.PMID: 14684380 [PubMed - indexed for MEDLINE]Related articlesFree article

Abnormal serotonergic development in a mouse model for the Smith-Lemli-Opitz syndrome: implications for autism.

Waage-Baudet H, Lauder JM, Dehart DB, Kluckman K, Hiller S, Tint GS, Sulik KK.

Int J Dev Neurosci. 2003 Dec;21(8):451-9.PMID: 14659996 [PubMed - indexed for MEDLINE]Related articles

Normal cognition and behavior in a Smith-Lemli-Opitz syndrome patient who presented with Hirschsprung disease.

Mueller C, Patel S, Irons M, Antshel K, Salen G, Tint GS, Bay C.

Am J Med Genet A. 2003 Nov 15;123A(1):100-6.PMID: 14556255 [PubMed - indexed for MEDLINE]Related articlesFree article

FXR-mediated down-regulation of CYP7A1 dominates LXRalpha in long-term cholesterol-fed NZW rabbits.

Xu G, Li H, Pan LX, Shang Q, Honda A, Ananthanarayanan M, Erickson SK, Shneider BL, Shefer S, Bollineni J, Forman BM, Matsuzaki Y, Suchy FJ, Tint GS, Salen G.

J Lipid Res. 2003 Oct;44(10):1956-62. Epub 2003 Aug 1.PMID: 12897188 [PubMed - indexed for MEDLINE]Related articlesFree article

Regulation of the farnesoid X receptor (FXR) by bile acid flux in rabbits.

Xu G, Pan LX, Li H, Forman BM, Erickson SK, Shefer S, Bollineni J, Batta AK, Christie J, Wang TH, Michel J, Yang S, Tsai R, Lai L, Shimada K, Tint GS, Salen G.

J Biol Chem. 2002 Dec 27;277(52):50491-6. Epub 2002 Oct 24.PMID: 12401785 [PubMed - indexed for MEDLINE]Related articlesFree article

Simultaneous quantitation of fatty acids, sterols and bile acids in human stool by capillary gas-liquid chromatography.

Batta AK, Salen G, Batta P, Tint GS, Alberts DS, Earnest DL.

J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 5;775(2):153-61.PMID: 12113981 [PubMed - indexed for MEDLINE]Related articles

Developmental sensitivity of associative learning to cholesterol synthesis inhibitors.

O'Brien WT, Xu G, Batta A, Tint GS, Salen G, Dyer CA, Kendler A, Servatius RJ.

Behav Brain Res. 2002 Feb 1;129(1-2):141-52.PMID: 11809505 [PubMed - indexed for MEDLINE]Related articles

Removal of the bile acid pool upregulates cholesterol 7alpha-hydroxylase by deactivating FXR in rabbits.

Xu G, Pan LX, Erickson SK, Forman BM, Shneider BL, Ananthanarayanan M, Li X, Shefer S, Balasubramanian N, Ma L, Asaoka H, Lear SR, Nguyen LB, Dussault I, Suchy FJ, Tint GS, Salen G.

J Lipid Res. 2002 Jan;43(1):45-50.PMID: 11792721 [PubMed - indexed for MEDLINE]Related articlesFree article

A cholesterol-lowering drug reduces beta-amyloid pathology in a transgenic mouse model of Alzheimer's disease.

Refolo LM, Pappolla MA, LaFrancois J, Malester B, Schmidt SD, Thomas-Bryant T, Tint GS, Wang R, Mercken M, Petanceska SS, Duff KE.

Neurobiol Dis. 2001 Oct;8(5):890-9.PMID: 11592856 [PubMed - indexed for MEDLINE]Related articles

Macrophage 3-hydroxy-3-methylglutaryl coenzyme a reductase activity in sitosterolemia: effects of increased cellular cholesterol and sitosterol concentrations.

Nguyen LB, Salen G, Shefer S, Tint GS, Ruiz F.

Metabolism. 2001 Oct;50(10):1224-9.PMID: 11586498 [PubMed - indexed for MEDLINE]Related articles

7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome.

Fitzky BU, Moebius FF, Asaoka H, Waage-Baudet H, Xu L, Xu G, Maeda N, Kluckman K, Hiller S, Yu H, Batta AK, Shefer S, Chen T, Salen G, Sulik K, Simoni RD, Ness GC, Glossmann H, Patel SB, Tint GS.

J Clin Invest. 2001 Sep;108(6):905-15.PMID: 11560960 [PubMed - indexed for MEDLINE]Related articlesFree article

Plant stanol fatty acid esters inhibit cholesterol absorption and hepatic hydroxymethyl glutaryl coenzyme A reductase activity to reduce plasma levels in rabbits.

Xu G, Salen G, Shefer S, Tint GS, Nguyen LB, Batta AK, Pcolinsky M.

Metabolism. 2001 Sep;50(9):1106-12.PMID: 11555847 [PubMed - indexed for MEDLINE]Related articles

Cholecystectomy prevents expansion of the bile acid pool and inhibition of cholesterol 7alpha-hydroxylase in rabbits fed cholesterol.

Xu G, Salen G, Shneider BL, Ananthanarayanan M, Shefer S, Ma L, Batta A, Nguyen LB, Lingutla JJ, Tint GS, Pcolinsky M, Suchy FJ.

J Lipid Res. 2001 Sep;42(9):1438-43.PMID: 11518763 [PubMed - indexed for MEDLINE]Related articlesFree article

Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis.

Honda A, Salen G, Matsuzaki Y, Batta AK, Xu G, Leitersdorf E, Tint GS, Erickson SK, Tanaka N, Shefer S.

J Biol Chem. 2001 Sep 14;276(37):34579-85. Epub 2001 Jul 13.PMID: 11454857 [PubMed - indexed for MEDLINE]Related articlesFree article

The effect of the erg26-1 mutation on the regulation of lipid metabolism in Saccharomyces cerevisiae.

Baudry K, Swain E, Rahier A, Germann M, Batta A, Rondet S, Mandala S, Henry K, Tint GS, Edlind T, Kurtz M, Nickels JT Jr.

J Biol Chem. 2001 Apr 20;276(16):12702-11. Epub 2001 Jan 18.PMID: 11279045 [PubMed - indexed for MEDLINE]Related articlesFree article

Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27(-/-) mice and CTX.

Honda A, Salen G, Matsuzaki Y, Batta AK, Xu G, Leitersdorf E, Tint GS, Erickson SK, Tanaka N, Shefer S.

J Lipid Res. 2001 Feb;42(2):291-300.PMID: 11181760 [PubMed - indexed for MEDLINE]Related articlesFree article

Mechanisms for cholesterol homeostasis in rat jejunal mucosa: effects of cholesterol, sitosterol, and lovastatin.

Nguyen LB, Shefer S, Salen G, Tint GS, Ruiz F, Bullock J.

J Lipid Res. 2001 Feb;42(2):195-200.PMID: 11181748 [PubMed - indexed for MEDLINE]Related articlesFree article

Hyperabsorption and retention of campestanol in a sitosterolemic homozygote: comparison with her mother and three control subjects.

Salen G, Xu G, Tint GS, Batta AK, Shefer S.

J Lipid Res. 2000 Nov;41(11):1883-9.PMID: 11060358 [PubMed - indexed for MEDLINE]Related articlesFree article

Blocking cholesterol synthesis impairs acquisition of the classically conditioned eyeblink response.

O'Brien WT, Xu G, Tint GS, Salen G, Servatius RJ.

Integr Physiol Behav Sci. 2000 Apr-Jun;35(2):120-31.PMID: 11021337 [PubMed - indexed for MEDLINE]Related articles

Hypercholesterolemia accelerates the Alzheimer's amyloid pathology in a transgenic mouse model.

Refolo LM, Malester B, LaFrancois J, Bryant-Thomas T, Wang R, Tint GS, Sambamurti K, Duff K, Pappolla MA.

Neurobiol Dis. 2000 Aug;7(4):321-31. Erratum in: Neurobiol Dis 2000 Dec;7(6 Pt B):690. PMID: 10964604 [PubMed - indexed for MEDLINE]Related articles

Is there a relationship between 3-hydroxy-3-methylglutaryl coenzyme a reductase activity and forebrain pathology in the PKU mouse?

Shefer S, Tint GS, Jean-Guillaume D, Daikhin E, Kendler A, Nguyen LB, Yudkoff M, Dyer CA.

J Neurosci Res. 2000 Sep 1;61(5):549-63.PMID: 10956425 [PubMed - indexed for MEDLINE]Related articles

Regulation of cholesterol biosynthetic pathway in patients with the Smith-Lemli-Opitz syndrome.

Honda M, Tint GS, Honda A, Salen G, Shefer S, Batta AK, Matsuzaki Y, Tanaka N.

J Inherit Metab Dis. 2000 Jul;23(5):464-74.PMID: 10947201 [PubMed - indexed for MEDLINE]Related articles

Abnormal hepatic sinusoidal bile acid transport in an Amish kindred is not linked to FIC1 and is improved by ursodiol.

Morton DH, Salen G, Batta AK, Shefer S, Tint GS, Belchis D, Shneider B, Puffenberger E, Bull L, Knisely AS.

Gastroenterology. 2000 Jul;119(1):188-95.PMID: 10889168 [PubMed - indexed for MEDLINE]Related articles

Spectrum of Delta(7)-dehydrocholesterol reductase mutations in patients with the Smith-Lemli-Opitz (RSH) syndrome.

Yu H, Lee MH, Starck L, Elias ER, Irons M, Salen G, Patel SB, Tint GS.

Hum Mol Genet. 2000 May 22;9(9):1385-91. Erratum in: Hum Mol Genet 2000 Jul 22;9(12):1903. PMID: 10814720 [PubMed - indexed for MEDLINE]Related articlesFree article

Fetal demise with Smith-Lemli-Opitz syndrome confirmed by tissue sterol analysis and the absence of measurable 7-dehydrocholesterol Delta(7)-reductase activity in chorionic villi.

Linck LM, Hayflick SJ, Lin DS, Battaile KP, Ginat S, Burlingame T, Gibson KM, Honda M, Honda A, Salen G, Tint GS, Connor WE, Steiner RD.

Prenat Diagn. 2000 Mar;20(3):238-40.PMID: 10719329 [PubMed - indexed for MEDLINE]Related articles

Detection of a common mutation in the RSH or Smith-Lemli-Opitz syndrome by a PCR-RFLP assay: IVS8-G-->C is found in over sixty percent of US propositi.

Yu H, Tint GS, Salen G, Patel SB.

Am J Med Genet. 2000 Feb 14;90(4):347-50.PMID: 10710236 [PubMed - indexed for MEDLINE]Related articles

Regulation of 25- and 27-hydroxylation side chain cleavage pathways for cholic acid biosynthesis in humans, rabbits, and mice. Assay of enzyme activities by high-resolution gas chromatography;-mass spectrometry.

Honda A, Salen G, Shefer S, Matsuzaki Y, Xu G, Batta AK, Tint GS, Tanaka N.

J Lipid Res. 2000 Mar;41(3):442-51.PMID: 10706592 [PubMed - indexed for MEDLINE]Related articlesFree article

3-Hydroxy-3-methylglutaryl-coenzyme A reductase activity is inhibited by cholesterol and up-regulated by sitosterol in sitosterolemic fibroblasts.

Honda A, Salen G, Honda M, Batta AK, Tint GS, Xu G, Chen TS, Tanaka N, Shefer S.

J Lab Clin Med. 2000 Feb;135(2):174-9.PMID: 10695663 [PubMed - indexed for MEDLINE]Related articles

Ileal bile acid transport regulates bile acid pool, synthesis, and plasma cholesterol levels differently in cholesterol-fed rats and rabbits.

Xu G, Shneider BL, Shefer S, Nguyen LB, Batta AK, Tint GS, Arrese M, Thevananther S, Ma L, Stengelin S, Kramer W, Greenblatt D, Pcolinsky M, Salen G.

J Lipid Res. 2000 Feb;41(2):298-304.PMID: 10681414 [PubMed - indexed for MEDLINE]Related articlesFree article

Comparative regulation of hepatic sterol 27-hydroxylase and cholesterol 7alpha-hydroxylase activities in the rat, guinea pig, and rabbit: effects of cholesterol and bile acids.

Nguyen LB, Xu G, Shefer S, Tint GS, Batta A, Salen G.

Metabolism. 1999 Dec;48(12):1542-8.PMID: 10599986 [PubMed - indexed for MEDLINE]Related articles

A convenient synthesis of dinorbile acids: oxidative hydrolysis of norbile acid nitriles.

Batta AK, Datta SC, Tint GS, Alberts DS, Earnest DL, Salen G.

Steroids. 1999 Nov;64(11):780-4.PMID: 10577835 [PubMed - indexed for MEDLINE]Related articles

Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver.

Honda A, Salen G, Shefer S, Batta AK, Honda M, Xu G, Tint GS, Matsuzaki Y, Shoda J, Tanaka N.

J Lipid Res. 1999 Aug;40(8):1520-8.PMID: 10428990 [PubMed - indexed for MEDLINE]Related articlesFree article

Antenatal therapy of Smith-Lemli-Opitz syndrome.

Irons MB, Nores J, Stewart TL, Craigo SD, Bianchi DW, D'Alton ME, Tint GS, Salen G, Bradley LA.

Fetal Diagn Ther. 1999 May-Jun;14(3):133-7.PMID: 10364662 [PubMed - indexed for MEDLINE]Related articles