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Related Concept Videos

Notch Signaling Pathway03:14

Notch Signaling Pathway

The Notch signaling pathway is a major intracellular signaling pathway that is highly conserved over a broad spectrum of metazoan species. It stands unique from other intracellular signaling mechanisms in animals because notch protein itself acts as the receptor as well as the primary signaling molecule.
The Notch gene came into the limelight in 1914 after the discovery that its mutation in Drosophila melanogaster leads to a serrated (or "notched") wing margin phenotype. It was not until 1985...
Notch Signaling Pathway03:14

Notch Signaling Pathway

The Notch signaling pathway is a major intracellular signaling pathway that is highly conserved over a broad spectrum of metazoan species. It stands unique from other intracellular signaling mechanisms in animals because notch protein itself acts as the receptor as well as the primary signaling molecule.
The Notch gene came into the limelight in 1914 after the discovery that its mutation in Drosophila melanogaster leads to a serrated (or "notched") wing margin phenotype. It was not until 1985...
Mechanism of Filopodia Formation01:39

Mechanism of Filopodia Formation

Filopodia are thin, actin-rich cellular protrusions that play an important role in many fundamental cellular functions. They vary in their occurrence, length, and positioning in different cell types, suggesting their diverse roles.
Their main function is to guide migrating cells during normal tissue morphogenesis or cancer metastasis by recognizing and making initial contacts with the extracellular matrix. However, they can also act as stationary cell anchors or help to establish communication...
Microtubules in Signaling01:22

Microtubules in Signaling

The primary cilium, made up of microtubules, acts as antennae on the cell surfaces for relaying external stimuli into the cells. These fine hair-like structures are present, generally one per cell. These are non-motile cilia in a 9+0 microtubules arrangement, where the central pair of microtubules are absent. The primary cilia arise from the basal body embedded in the cell membrane. Intraflagellar transport (IFT) carries requisite proteins from the cytoplasm to the cilium because the primary...
Role Of Notch Signalling In Intestinal Stem Cell Renewal01:12

Role Of Notch Signalling In Intestinal Stem Cell Renewal

Notch signaling was first discovered in Drosophila melanogaster, where it is involved in cell lineage differentiation. Notch signaling regulates the maintenance and differentiation of intestinal stem cells or ISCs by controlling the expression of atonal homolog 1 or Atoh1. Atoh1 directs cells to differentiate into secretory cells.
Direct cell-to-cell contact is needed for the activation of Notch signaling. The signal is initiated when a notch ligand binds to a receptor on an adjacent cell, also...
Positive Regulator Molecules02:39

Positive Regulator Molecules

Mitotic cell division results in daughter cells that exactly resemble the parent cell. However, errors in the DNA replication or distribution of genetic material may lead to genetic mutations that may be passed down to every new cell formed from the resulting abnormal cell. Propagation of such mutant cells is restricted through checkpoint mechanisms present at different stages of the cell cycle. These checkpoints involve regulator molecules that either promote or demote cell cycle events.

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Related Experiment Video

Updated: Jul 15, 2026

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis
07:26

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis

Published on: April 1, 2022

Polyductin undergoes notch-like processing and regulated release from primary cilia.

Jun-ya Kaimori1, Yasuyuki Nagasawa, Luis F Menezes

  • 1Division of Nephrology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Human Molecular Genetics
|May 2, 2007
PubMed
Summary

The PKHD1 gene product, polyductin/fibrocystin, undergoes shedding from primary cilia via proteolytic processing. This shedding mechanism suggests a novel signaling role for ciliary proteins in autosomal recessive polycystic kidney disease (ARPKD).

More Related Videos

Cell Aggregation Assays to Evaluate the Binding of the Drosophila Notch with Trans-Ligands and its Inhibition by Cis-Ligands
05:48

Cell Aggregation Assays to Evaluate the Binding of the Drosophila Notch with Trans-Ligands and its Inhibition by Cis-Ligands

Published on: January 2, 2018

Simple Detection of Primary Cilia by Immunofluorescence
08:07

Simple Detection of Primary Cilia by Immunofluorescence

Published on: May 15, 2020

Related Experiment Videos

Last Updated: Jul 15, 2026

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis
07:26

Isolation of Whole Cell Protein Lysates from Mouse Facial Processes and Cultured Palatal Mesenchyme Cells for Phosphoprotein Analysis

Published on: April 1, 2022

Cell Aggregation Assays to Evaluate the Binding of the Drosophila Notch with Trans-Ligands and its Inhibition by Cis-Ligands
05:48

Cell Aggregation Assays to Evaluate the Binding of the Drosophila Notch with Trans-Ligands and its Inhibition by Cis-Ligands

Published on: January 2, 2018

Simple Detection of Primary Cilia by Immunofluorescence
08:07

Simple Detection of Primary Cilia by Immunofluorescence

Published on: May 15, 2020

Area of Science:

  • Cell Biology
  • Molecular Biology
  • Genetics

Background:

  • Mutations in the PKHD1 gene cause autosomal recessive polycystic kidney disease (ARPKD).
  • The role of the PKHD1 gene product, polyductin/fibrocystin, in planar cell polarity and ARPKD pathogenesis is unclear.
  • The 4074 amino acid ciliary protein's function in regulating cellular processes remains largely unknown.

Purpose of the Study:

  • To investigate the processing and localization of the PKHD1 gene product, polyductin/fibrocystin.
  • To elucidate the mechanism by which polyductin/fibrocystin interacts with primary cilia.
  • To explore the potential signaling functions of ciliary proteins in ARPKD.

Main Methods:

  • Utilized novel in vitro expression systems to study polyductin/fibrocystin processing.
  • Analyzed proteolytic cleavage sites, including proprotein convertase and gamma-secretase dependent pathways.
  • Investigated the role of ADAM metalloproteinase disintegrins family members in protein shedding.

Main Results:

  • Polyductin/fibrocystin undergoes complex, Notch-like proteolytic processing, involving cleavage and shedding of its extracellular domain from primary cilia.
  • ADAM metalloproteinase disintegrins and gamma-secretase are involved in the shedding of polyductin/fibrocystin's extracellular and intracellular fragments.
  • Endogenous polyductin/fibrocystin ectodomain shedding from primary cilia is regulated by sheddase activation.

Conclusions:

  • This study reveals a novel mechanism of regulated protein shedding from primary cilia, involving polyductin/fibrocystin.
  • The shedding process suggests that primary ciliary proteins can act as bi-directional signaling molecules.
  • Regulated release into the lumen may serve as a mechanism for distributing signals to downstream targets via flow, potentially impacting ARPKD.