Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Mechanism of Ciliary Motion01:05

Mechanism of Ciliary Motion

5.2K
The ciliary structures were first seen in 1647 by Antonie Leeuwenhoek while observing the protozoans. In lower organisms, these appendages are responsible for cell movement, while in higher organisms, these appendages help in the movement of the extracellular fluids within the body cavities.
The cilia are made up of microtubules in a 9+2 arrangement, with nine microtubule doublet ring bundles, surrounding a pair of central singlet microtubule bundles. The doublet microtubule bundles are...
5.2K
Sustainable Development01:43

Sustainable Development

15.2K
As the human population continues to grow and use resources, we must be mindful of our planet’s natural limits. Sustainable development provides a pathway to maintain and improve human life now while also ensuring that future generations will have the resources that they need. The long-term success of sustainability efforts rests on understanding the interplay between human actions and ecological systems.
15.2K
Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

1.1K
Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
1.1K
Protein-protein Interfaces02:04

Protein-protein Interfaces

14.7K
Many proteins form complexes to carry out their functions, making protein-protein interactions (PPIs) essential for an organism's survival. Most PPIs are stabilized by numerous weak noncovalent chemical forces. The physical shape of the interfaces determines the way two proteins interact. Many globular proteins have closely-matching shapes on their surfaces, which form a large number of weak bonds. Additionally, many PPIs occur between two helices or between a surface cleft and a...
14.7K
Protein and Protein Structure02:15

Protein and Protein Structure

88.2K
Proteins are one of the most abundant organic molecules in living systems and have the most diverse range of functions of all macromolecules. Proteins may be structural, regulatory, contractile, or protective. They may serve in transport, storage, or membranes; or they may be toxins or enzymes. Their structures, like their functions, vary greatly. They are all, however, amino acid polymers arranged in a linear sequence.
A protein's shape is critical to its function. For example, an enzyme...
88.2K
Development of the Lymphatic System01:15

Development of the Lymphatic System

2.1K
The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
2.1K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A goat-derived gamma-tubulin antibody for triple-channel imaging of primary cilia.

BMC molecular and cell biology·2026
Same author

Oxidative stress causes a reversible decrease of deubiquitylases activity in old vertebrate brains.

Nature communications·2026
Same author

In situ proximity ligation assay for analysing spatial interactions between ciliary proteins.

BMC molecular and cell biology·2025
Same author

A differential requirement for ciliary transition zone proteins in human and mouse neural progenitor fate specification.

Nature communications·2025
Same author

Editorial: Regulation of proteostasis and cellular energy homeostasis at the primary cilium.

Frontiers in cell and developmental biology·2023
Same author

A Systematic Review on the Visualization of Avatars and Agents in AR & VR displayed using Head-Mounted Displays.

IEEE transactions on visualization and computer graphics·2023

Related Experiment Video

Updated: Feb 7, 2026

Isolation and Culture of Chick Ciliary Ganglion Neurons
14:36

Isolation and Culture of Chick Ciliary Ganglion Neurons

Published on: August 8, 2020

6.3K

The ciliary protein Rpgrip1l in development and disease.

Antonia Wiegering1, Ulrich Rüther1, Christoph Gerhardt1

  • 1Institute for Animal Developmental and Molecular Biology, Heinrich Heine University Düsseldorf, 40225 Düsseldorf, Germany.

Developmental Biology
|August 4, 2018
PubMed
Summary

RPGRIP1L protein dysfunction causes ciliopathies, impacting multiple organ development. Research in model organisms reveals novel gene functions and potential therapeutic strategies for these severe human diseases.

Keywords:
AutophagyCiliaCiliopathiesFtmProteasomeRpgrip1Transition zone

More Related Videos

Observation of the Ciliary Movement of Choroid Plexus Epithelial Cells Ex Vivo
08:00

Observation of the Ciliary Movement of Choroid Plexus Epithelial Cells Ex Vivo

Published on: July 13, 2015

12.7K
High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
05:32

High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia

Published on: January 19, 2022

5.1K

Related Experiment Videos

Last Updated: Feb 7, 2026

Isolation and Culture of Chick Ciliary Ganglion Neurons
14:36

Isolation and Culture of Chick Ciliary Ganglion Neurons

Published on: August 8, 2020

6.3K
Observation of the Ciliary Movement of Choroid Plexus Epithelial Cells Ex Vivo
08:00

Observation of the Ciliary Movement of Choroid Plexus Epithelial Cells Ex Vivo

Published on: July 13, 2015

12.7K
High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
05:32

High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia

Published on: January 19, 2022

5.1K

Area of Science:

  • Molecular Biology
  • Genetics
  • Developmental Biology

Background:

  • RPGRIP1L is a highly conserved gene crucial for primary cilia function.
  • Mutations in RPGRIP1L lead to ciliopathies, a class of severe human genetic disorders.
  • Ciliopathies are associated with impaired development across multiple organs.

Purpose of the Study:

  • To elucidate the role of RPGRIP1L in human development.
  • To understand the molecular mechanisms underlying RPGRIP1L-associated ciliopathies.
  • To review novel functions of RPGRIP1L and explore therapeutic approaches.

Main Methods:

  • Review of existing literature on RPGRIP1L.
  • Analysis of findings from various model organisms.
  • Synthesis of research on ciliopathies and potential treatments.

Main Results:

  • RPGRIP1L protein localizes to the primary cilia transition zone.
  • Dysfunctional RPGRIP1L disrupts cilia, leading to multi-organ developmental defects.
  • Investigations have identified new functions of RPGRIP1L and promising therapeutic avenues.

Conclusions:

  • RPGRIP1L is essential for normal human development via its role in primary cilia.
  • Understanding RPGRIP1L function is key to addressing ciliopathies.
  • Further research may lead to effective treatments for patients with RPGRIP1L mutations.