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

Journal of lipid research, 2006-07, Vol.47 (7), p.1535-1541

2006

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A Cholesterol-Lowering Drug Reduces β-Amyloid Pathology in a Transgenic Mouse Model of Alzheimer's Disease

Neurobiology of disease, 2001-10, Vol.8 (5), p.890-899

2001

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

Journal of lipid research, 2004-11, Vol.45 (11), p.2044-2051

2004

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Defective Cholesterol Biosynthesis Associated with the Smith-Lemli-Opitz Syndrome

The New England journal of medicine, 1994-01, Vol.330 (2), p.107-113

1994

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Sitosterolemia

Journal of lipid research, 1992-07, Vol.33 (7), p.945-955

1992

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Correlation of severity and outcome with plasma sterol levels in variants of the Smith-Lemli-Opitz syndrome

The Journal of pediatrics, 1995-07, Vol.127 (1), p.82-87

1995

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

American journal of medical genetics, 2003-11, Vol.123A (1), p.100-106

2003

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

International journal of developmental neuroscience, 2003-12, Vol.21 (8), p.451-459

2003

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Hypercholesterolemia Accelerates the Alzheimer's Amyloid Pathology in a Transgenic Mouse Model

Neurobiology of disease, 2000-08, Vol.7 (4), p.321-331

2000

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Genetic Evidence for Nonredundant Functional Cooperativity between NPC1 and NPC2 in Lipid Transport

Proceedings of the National Academy of Sciences - PNAS, 2004-04, Vol.101 (16), p.5886-5891

2004

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

Journal of lipid research, 2001-02, Vol.42 (2), p.291-300

2001

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Markedly increased tissue concentrations of 7-dehydrocholesterol combined with low levels of cholesterol are characteristic of the Smith-Lemli-Opitz syndrome

Journal of lipid research, 1995-01, Vol.36 (1), p.89-95

1995

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Desmosterol in Brain Is Elevated because DHCR24 Needs REST for Robust Expression but REST Is Poorly Expressed

Developmental neuroscience, 2014-01, Vol.36 (2), p.132-142

2014

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Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann–Pick type C phenotype

Journal of inherited metabolic disease, 1998-12, Vol.21 (8), p.853-863

1998

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Abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome

Journal of lipid research, 1996-06, Vol.37 (6), p.1169-1180

1996

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Developmental sensitivity of associative learning to cholesterol synthesis inhibitors

Behavioural brain research, 2002-02, Vol.129 (1), p.141-152

2002

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Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome

Journal of lipid research, 1995-04, Vol.36 (4), p.705-713

1995

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

Journal of lipid research, 2000-11, Vol.41 (11), p.1883-1889

2000

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Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes

Journal of lipid research, 1995-07, Vol.36 (7), p.1595-1601

1995

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7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts

Journal of lipid research, 1998-03, Vol.39 (3), p.647-657

1998

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

Journal of lipid research, 2001-02, Vol.42 (2), p.195-200

2001

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Differing effects of cholesterol and taurocholate on steady state hepatic HMG-CoA reductase and cholesterol 7 alpha-hydroxylase activities and mRNA levels in the rat

Journal of lipid research, 1992-08, Vol.33 (8), p.1193-1200

1992

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Increased bile acid pool inhibits cholesterol 7 alpha-hydroxylase in cholesterol-fed rabbits

Gastroenterology (New York, N.Y. 1943), 1997-12, Vol.113 (6), p.1958-1965

1997

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

Gastroenterology (New York, N.Y. 1943), 2000-07, Vol.119 (1), p.188-195

2000

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Increased sitosterol absorption, decreased removal, and expanded body pools compensate for reduced cholesterol synthesis in sitosterolemia with xanthomatosis

Journal of lipid research, 1989-09, Vol.30 (9), p.1319-1330

1989

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

A Cholesterol-Lowering Drug Reduces β-Amyloid Pathology in a Transgenic Mouse Model of Alzheimer's Disease

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

Defective Cholesterol Biosynthesis Associated with the Smith-Lemli-Opitz Syndrome

Sitosterolemia

Correlation of severity and outcome with plasma sterol levels in variants of the Smith-Lemli-Opitz syndrome

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

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

Hypercholesterolemia Accelerates the Alzheimer's Amyloid Pathology in a Transgenic Mouse Model

Genetic Evidence for Nonredundant Functional Cooperativity between NPC1 and NPC2 in Lipid Transport

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

Markedly increased tissue concentrations of 7-dehydrocholesterol combined with low levels of cholesterol are characteristic of the Smith-Lemli-Opitz syndrome

Desmosterol in Brain Is Elevated because DHCR24 Needs REST for Robust Expression but REST Is Poorly Expressed

Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann–Pick type C phenotype

Abnormal cholesterol biosynthesis in the Smith-Lemli-Opitz syndrome

Developmental sensitivity of associative learning to cholesterol synthesis inhibitors

Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome

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

Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes

7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts

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

Differing effects of cholesterol and taurocholate on steady state hepatic HMG-CoA reductase and cholesterol 7 alpha-hydroxylase activities and mRNA levels in the rat

Increased bile acid pool inhibits cholesterol 7 alpha-hydroxylase in cholesterol-fed rabbits

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

Increased sitosterol absorption, decreased removal, and expanded body pools compensate for reduced cholesterol synthesis in sitosterolemia with xanthomatosis

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