Hedgehog Signaling Pathway Studies

HES1 is a critical mediator of the SHH-GLI3 axis in regulating digit number

The paper identifies HES1 as a critical mediator of the SHH-GLI3 axis in regulating digit number, showing its role in inducing polydactyly and modulating cell proliferation and chondrogenesis.

Heterozygous mutation of Sonic Hedgehog receptor (Ptch) drives cerebellar overgrowth and sex-specifically alters hippocampal and cortical layer structure, activity, and social behavior in female mice

This study shows that heterozygous Ptch1 mutation in mice causes cerebellar overgrowth and sex-specific changes in hippocampal and cortical structure, activity, and social behavior.

GLI transcriptional repression regulates tissue-specific enhancer activity in response to Hedgehog signaling

The paper identifies HH-responsive enhancers that lose acetylation in the absence of Hedgehog signaling and are regulated by GLI repression rather than Polycomb complexes.

Ciliary phosphatidylinositol phosphatase Inpp5e plays positive and negative regulatory roles in Shh signaling

The paper identifies Inpp5e's dual role in Shh signaling by showing it regulates GliA and GliR timing, affecting neural tube patterning.

Neural tube development depends on notochord-derived Sonic hedgehog released into the sclerotome

The paper shows that Shh from the sclerotome affects neural tube development by acting on the basal side of the neuroepithelium, influencing motoneuron numbers and myotome differentiation.

The Hedgehog Co-Receptor BOC Differentially Regulates SHH Signaling During Craniofacial Development

This study identifies dual, tissue-specific roles for the Hedgehog co-receptor BOC in promoting and antagonizing HH signaling during craniofacial development.

Association of Sonic Hedgehog with the Extracellular Matrix Requires its Putative Zinc-Peptidase Activity

This study shows that Sonic Hedgehog's zinc-coordination fold is necessary for its association with the extracellular matrix and non-cell autonomous signaling.

Endosome-Mediated Epithelial Remodeling Downstream of Hedgehog/Gli Is Required for Tracheoesophageal Separation

This paper identifies HH/Gli-dependent cellular mechanisms, including endosome-mediated remodeling and matrix degradation, essential for tracheoesophageal separation in mouse and Xenopus embryos.

Notch signalling maintains Hedgehog responsiveness via a Gli-dependent mechanism during spinal cord patterning in zebrafish

The paper investigates how Notch signalling maintains Hedgehog responsiveness in zebrafish spinal cord development through a Gli-dependent mechanism.

The effect of sonic hedgehog on motor neuron positioning in the spinal cord during chicken embryo development

This study shows that overexpression of Sonic hedgehog in chicken embryos disrupts motor neuron positioning and motor column structure by affecting transcription factors and axon projections.

A novel truncating variant of GLI2 associated with Culler-Jones syndrome impairs Hedgehog signalling

The paper identifies a novel truncating GLI2 variant linked to Culler-Jones syndrome and demonstrates its dominant-negative effect on Hedgehog signaling through functional assays.

Dynamic Hh signaling can generate temporal information during tissue patterning

The paper shows that Hh signaling in Drosophila ocellar retina uses a non-linear gradient as a temporal cue to generate a wave of differentiating photoreceptors at constant speed.

GPR17 is an Essential Component of the Negative Feedback Loop of the Sonic Hedgehog Signalling Pathway in Neural Tube Development

This study identifies GPR17 as a negative regulator of the Sonic Hedgehog signaling pathway in neural tube development, showing its role in temporal regulation and feedback inhibition.

Gain-of-function Shh mutants activate Smo in cis independent of Ptch1/2 function

The paper identifies Shh mutations that enable cell-autonomous activation of the Hh pathway via Smo, independent of Ptch1/2, and shows that unprocessed Shh pro-peptide can drive this activation.

SOX2 Regulation by Hedgehog Signaling Controls Adult Lingual Epithelium Homeostasis

This study shows that SOX2, regulated by Hedgehog signaling, is essential for maintaining adult lingual epithelium homeostasis, including taste bud differentiation and epithelial cell balance.

Distinct temporal requirements for Sonic hedgehog signaling in development of the tuberal hypothalamus

This study defines the distinct temporal requirements for Sonic hedgehog signaling in tuberal hypothalamic development using fate-mapping and conditional deletion in mice.

Sonic hedgehog is required for neural crest-dependent patterning of the intrinsic tongue musculature

This study shows that Sonic hedgehog (Shh) is essential for patterning the intrinsic tongue muscles through cranial neural crest-derived mesenchyme in mice.