Cyclopamine Inhibition of Sonic Hedgehog Signal Transduction Is Not Mediated through Effects on Cholesterol Transport

Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function

1996 • 3,041 citations

Niemann-Pick C1 Disease Gene: Homology to Mediators of Cholesterol Homeostasis

1997 • 1,440 citations

Mutations in the human Sonic Hedgehog gene cause holoprosencephaly

1996 • 1,140 citations

The tumour-suppressor gene patched encodes a candidate receptor for Sonic hedgehog

1996 • 1,106 citations

Pax6 Controls Progenitor Cell Identity and Neuronal Fate in Response to Graded Shh Signaling

1997 • 1,019 citations

Floor plate and motor neuron induction by different concentrations of the amino-terminal cleavage product of sonic hedgehog autoproteolysis

1995 • 886 citations

Biochemical evidence that Patched is the Hedgehog receptor

1996 • 852 citations

Teratogen-Mediated Inhibition of Target Tissue Response to Shh Signaling

1998 • 839 citations

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

1994 • 802 citations

Murine Model of Niemann-Pick C Disease: Mutation in a Cholesterol Homeostasis Gene

1997 • 797 citations

A lipid associated with the antiphospholipid syndrome regulates endosome structure and function

1998 • 774 citations

Identification of a Palmitic Acid-modified Form of Human Sonic hedgehog

1998 • 735 citations

Diversity and Pattern in the Developing Spinal Cord

1996 • 714 citations

Mouse Gli1 mutants are viable but have defects in SHH signaling in combination with a Gli2 mutation

2000 • 704 citations

The teratogenic Veratrum alkaloid cyclopamine inhibits Sonic hedgehog signal transduction

1998 • 647 citations

Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport

1999 • 628 citations

Maternal Pumilio acts together with Nanos in germline development in Drosophila embryos

1999 • 527 citations

Gli2 is required for induction of floor plate and adjacent cells, but not most ventral neurons in the mouse central nervous system

1998 • 497 citations

Essential Role forSonic hedgehogduring Hair Follicle Morphogenesis

1999 • 489 citations

Sterol Resistance in CHO Cells Traced to Point Mutation in SREBP Cleavage–Activating Protein

1996 • 473 citations

Control of cell pattern in the neural tube: Motor neuron induction by diffusible factors from notochord and floor plate

1993 • 470 citations

Mammalian Suppressor-of-Fused modulates nuclear–cytoplasmic shuttling of GLI-1

1999 • 454 citations

Diminished Sonic hedgehog signaling and lack of floor plate differentiation in Gli2 mutant mice

1998 • 448 citations

Transducing Hedgehog: the story so far

1998 • 444 citations

Intracellular cholesterol transport

1999 • 416 citations

Endocytic Sorting of Lipid Analogues Differing Solely in the Chemistry of Their Hydrophobic Tails

1999 • 378 citations

The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo

1999 • 375 citations

Mutations in the Δ7-sterol reductase gene in patients with the Smith–Lemli–Opitz syndrome

1998 • 371 citations

Hedgehog Elicits Signal Transduction by Means of a Large Complex Containing the Kinesin-Related Protein Costal2

1997 • 371 citations

Costal2, a Novel Kinesin-Related Protein in the Hedgehog Signaling Pathway

1997 • 329 citations

Sonic hedgehog signaling by the Patched–Smoothened receptor complex

1999 • 326 citations

The intracellular transport of low density lipoprotein-derived cholesterol is inhibited in Chinese hamster ovary cells cultured with 3-β-[2-(diethylamino)ethoxy]androst-5-en-17-one

1989 • 315 citations

Cholesterol-dependent retention of GPI-anchored proteins in endosomes

1998 • 313 citations

Cooperation of BMP7 and SHH in the Induction of Forebrain Ventral Midline Cells by Prechordal Mesoderm

1997 • 292 citations

Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases

1999 • 288 citations

Characterization of two patched receptors for the vertebrate hedgehog protein family

1998 • 281 citations

Pancreas development is promoted by cyclopamine, a Hedgehog signaling inhibitor

1998 • 256 citations

The specification of neuronal identity by graded sonic hedgehog signalling

1999 • 252 citations

Niemann–Pick C1 Is a Late Endosome-Resident Protein That Transiently Associates with Lysosomes and the Trans-Golgi Network

1999 • 252 citations

Holoprosencephaly in RSH/Smith-Lemli-Opitz syndrome: Does abnormal cholesterol metabolism affect the function ofsonic hedgehog?

1996 • 219 citations

Mouse Suppressor of fused is a negative regulator of Sonic hedgehog signaling and alters the subcellular distribution of Gli1

1999 • 203 citations

Type-C Niemann-Pick disease: low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes.

1988 • 203 citations

Characterization of the human Suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli

1999 • 184 citations

Holoprosencephaly: A paradigm for the complex genetics of brain development

1998 • 176 citations

In vivo evidence that Patched and Smoothened constitute distinct binding and transducing components of a Hedgehog receptor complex

1998 • 173 citations

Progesterone blocks cholesterol translocation from lysosomes.

1992 • 170 citations

Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

1999 • 161 citations

Mediation of Sonic Hedgehog-Induced Expression of COUP-TFII by a Protein Phosphatase

1997 • 149 citations

Vertebrate Homologs of Drosophila Suppressor of Fused Interact with the Gli Family of Transcriptional Regulators

1999 • 127 citations

Transient establishment of anteroposterior polarity in the zebrafish pectoral fin bud in the absence of sonic hedgehog activity

1999 • 122 citations

Cholesterol homeostasis. Modulation by amphiphiles.

1994 • 120 citations

Subcellular localization of the segment polarity protein patched suggests an interaction with the wingless reception complex in Drosophila embryos

1994 • 111 citations

Role of the plasma membrane in cholesterol esterification in rat hepatoma cells

1993 • 102 citations

Molecular Dissection of the Role of the Membrane Domain in the Regulated Degradation of 3-Hydroxy-3-methylglutaryl Coenzyme A Reductase

1995 • 99 citations

Type C Niemann-Pick Disease: Use of Hydrophobic Amines to Study Defective Cholesterol Transport

1991 • 97 citations

Abnormal cholesterol metabolism in imipramine-treated fibroblast cultures. Similarities with Niemann-Pick type C disease

1990 • 93 citations

Quantitative analysis of hydrophobic amine inhibition of intracellular cholesterol transport.

1996 • 84 citations

Cholesterol Biosynthesis from Lanosterol

1999 • 84 citations

Free sphingoid bases in tissues from patients with type C Niemann-Pick disease and other lysosomal storage disorders

1994 • 83 citations

Complex formation between solanaceous steroidal glycoalkaloids and free sterols in vitro

1979 • 78 citations

Inhibition of 7-dehydrocholesterol reductase by the teratogen AY9944: A rat model for Smith-Lemli-Opitz syndrome

1996 • 65 citations

Mobilization of late-endosomal cholesterol is inhibited by Rab guanine nucleotide dissociation inhibitor

2000 • 63 citations

Identification of a Novel Sonic Hedgehog Response Element in the Chicken Ovalbumin Upstream Promoter-Transcription Factor II Promoter

1997 • 55 citations

Prenatal diagnosis of Niemann-Pick type C disease: current strategy from an experience of 37 pregnancies at risk.

1992 • 55 citations

NPC1-Containing Compartment of Human Granulosa-Lutein Cells: A Role in the Intracellular Trafficking of Cholesterol Supporting Steroidogenesis

2000 • 52 citations

Holoprosencephaly: An Overview and Atlas of Cases

1990 • 43 citations

Steroidal Alkaloid Teratogens: Molecular Probes for Investigation of Craniofacial Malformations

1996 • 41 citations

Carbon-13 and Proton Nuclear Magnetic Resonance Spectra of Veratrum Alkaloids

1986 • 31 citations

Implication of C-5, C-6 unsaturation as a key structural factor in steroidal alkaloid-induced mammalian teratogenesis

1993 • 29 citations

Structure-activity relation of steroid teratogens. 1. Jervine ring system

1978 • 28 citations

Sterol-mediated regulation of mevalonic acid synthesis. Accumulation of 4-carboxysterols as the predominant sterols synthesized in a Chinese hamster ovary cell cholesterol auxotroph (mutant 215).

1990 • 23 citations

Effects of drugs on cholesterol esterification in normal and Niemann-Pick type C fibroblasts: AY-9944, other cationic amphiphilic drugs and DMSO

1991 • 22 citations

Mobilization of late-endosomal cholesterol is inhibited by Rab guanine nucleotide dissociation inhibitor

2000 • 21 citations

Fetal Niemann-Pick disease type C: Ultrastructural and lipid findings in liver and spleen

1993 • 18 citations

Structure-activity relation of steroid teratogens. 2. N-Substituted jervines

1978 • 14 citations

Prenatal diagnosis of Niemann-Pick disease type C

1992 • 12 citations

Isopentenoid Synthesis in Eukaryotic Cells

1993 • 9 citations

Holoprosencephaly — An overview and atlas of cases

1991 • 8 citations

Environmental Influences on Cells in Culture

2003 • 6 citations