Cited by 860Hedgehog Signaling Pathway StudiesPharmacological Effects and Toxicity StudiesGenetic and Kidney Cyst Diseases

Teratogen-Mediated Inhibition of Target Tissue Response to Shh Signaling

Cooper, Michael K., Porter, Jeffery A., Young, Keith E., Beachy, Philip A.·1998·Science

Abstract

Veratrum alkaloids and distal inhibitors of cholesterol biosynthesis have been studied for more than 30 years as potent teratogens capable of inducing cyclopia and other birth defects. Here, it is shown that these compounds specifically block the Sonic hedgehog (Shh) signaling pathway. These teratogens did not prevent the sterol modification of Shh during autoprocessing but rather inhibited the response of target tissues to Shh, possibly acting through the sterol sensing domain within the Patched protein regulator of Shh response.

Integrity signals

Registry

Crossref · 10.1126/science.280.5369.1603

Cites

26 of 26 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Low cholesterol levels may impair sonic hedgehog signaling and cause the striking developmental malformations in SLOS.

    “Low cholesterol levels may impair sonic hedgehog signaling and cause the striking developmental malformations. Activity of this signaling factor and its distribution are regulated by posttranslational attachment of cholesterol to its amino terminus [26,27,28].”

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Authors
Cooper, M. K., Porter, J. A., Young, K. E., & Beachy, P. A.
Affiliations
Howard Hughes Medical Institute (US)
Corresponding author
  • Porter, Jeffery A.
  • Beachy, Philip A.
Venue
Science
Published
1998-06-05
Volume
280
Issue
5369
Pages
1603-1607
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2002249795
Cited by
848
References
26
Citations by year
2026: 7 · 2025: 7 · 2024: 12 · 2023: 13 · 2022: 18 · 2021: 9 · 2020: 16 · 2019: 25 · 2018: 22 · 2017: 25 · 2016: 26 · 2015: 27 · 2014: 34 · 2013: 31 · 2012: 50
FWCI
14.39
Type
journal article
Language
en
Open access
closed
Locations
  • Science (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Hedgehog Signaling Pathway Studies
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pharmacological Effects and Toxicity Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
Keywords
teratogens, Sonic hedgehog signaling, veratrum alkaloids, cholesterol biosynthesis inhibitors, cyclopia, congenital malformations, sterol-sensing domain
MeSH terms
Abnormalities, Drug-Induced, Patched Receptors, Animals, trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride, Cell Membrane, Central Nervous System, Chick Embryo, Cholesterol, DNA-Binding Proteins, Endoplasmic Reticulum, Membrane Proteins, Muscle Proteins, Nerve Tissue Proteins, Nuclear Proteins, Proteins, Receptors, Cell Surface, Teratogens, Tomatine, Transcription Factors, Triparanol, Veratrum Alkaloids, Signal Transduction, Trans-Activators, Holoprosencephaly, Homeodomain Proteins, Culture Techniques, Hepatocyte Nuclear Factor 3-beta, PAX7 Transcription Factor, Hedgehog Proteins, LIM-Homeodomain Proteins

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review