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Hedgehog Signaling Pathway

Hedgehog Signaling Pathway

The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...

In-vitro Mutagenesis

In-vitro Mutagenesis

To learn more about the function of a gene, researchers can observe what happens when the gene is inactivated or “knocked out,” by creating genetically engineered knockout animals. Knockout mice have been particularly useful as models for human diseases such as cancer, Parkinson’s disease, and diabetes.

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Related Experiment Video

Updated: Jan 13, 2026

A Phenotyping Regimen for Genetically Modified Mice Used to Study Genes Implicated in Human Diseases of Aging

A Phenotyping Regimen for Genetically Modified Mice Used to Study Genes Implicated in Human Diseases of Aging

Published on: July 14, 2016

Dissecting Hhip1 Function In Vivo Using a Conditional Knockout Mouse Model.

Ryuma Haraguchi¹, Riko Kitazawa^1,2, Yuta Yanagihara³

¹Department of Molecular Pathology, Ehime University Graduate School of Medicine, Shitsukawa, Toon City, Ehime 791-0295, Japan.

Acta Histochemica Et Cytochemica

|January 9, 2026

Summary

This summary is machine-generated.

A new conditional knockout mouse model for Hedgehog-interacting protein 1 (Hhip1) reveals its critical role in maintaining postnatal growth plate architecture and preventing long bone overgrowth.

Keywords:

Hhip1 conditional knockout hedgehog signaling long bone development lung morphogenesis

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Last Updated: Jan 13, 2026

A Phenotyping Regimen for Genetically Modified Mice Used to Study Genes Implicated in Human Diseases of Aging

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Published on: July 14, 2016

Ex Utero Electroporation and Organotypic Slice Culture of Mouse Hippocampal Tissue

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Published on: March 4, 2015

Skeletal Phenotype Analysis of a Conditional Stat3 Deletion Mouse Model

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Published on: July 3, 2020

Area of Science:

Developmental Biology
Genetics
Molecular Signaling

Background:

The Hedgehog (Hh) signaling pathway is crucial for development and homeostasis.
Hedgehog-interacting protein 1 (Hhip1) is a negative regulator of Hh signaling.
Previous studies were limited by perinatal lethality in global Hhip1 knockout models.

Purpose of the Study:

To develop a conditional knockout model for studying postnatal Hhip1 functions.
To investigate the role of Hhip1 in postnatal development, particularly in limb development.
To explore the spatiotemporal regulation of Hh signaling.

Main Methods:

Generation of a conditional Hhip1 allele (Hhip1^flox) using CRISPR/Cas9.
Cre-mediated excision to create a conditional null allele (Hhip1^ex2Δ).
Utilized CMV-Cre for germline recombination and Prx1-Cre for limb-specific knockouts.

Main Results:

The Hhip1^flox model successfully recapitulated known Hhip1 knockout phenotypes.
Limb-specific Hhip1 conditional knockout mice showed progressive growth plate expansion and long bone overgrowth.
Sustained upregulation of Gli1 expression was observed in conditional knockout mice.

Conclusions:

The Hhip1^flox model is a valuable tool for tissue- and stage-specific functional studies of Hhip1.
Hhip1 plays a previously unrecognized role in maintaining postnatal growth plate architecture.
This model provides insights into Hh signaling regulation in development and disease.