Antennas of organ morphogenesis: the roles of cilia in vertebrate kidney development.

Genesis

Department of Biological Sciences, Center for Stem Cells and Regenerative Medicine, Center for Zebrafish Research, University of Notre Dame, Notre Dame, IN, 46556, USA.

Published: September 2016

Cilia arose early during eukaryotic evolution, and their structural components are highly conserved from the simplest protists to complex metazoan species. In recent years, the role of cilia in the ontogeny of vertebrate organs has received increasing attention due to a staggering correlation between human disease and dysfunctional cilia. In particular, the presence of cilia in both the developing and mature kidney has become a deep area of research due to ciliopathies common to the kidney, such as polycystic kidney disease (PKD). Interestingly, mutations in genes encoding proteins that localize to the cilia cause similar cystic phenotypes in kidneys of various vertebrates, suggesting an essential role for cilia in kidney organogenesis and homeostasis as well. Importantly, the genes so far identified in kidney disease have conserved functions across species, whose kidneys include both primary and motile cilia. Here, we aim to provide a comprehensive description of cilia and their role in kidney development, as well as highlight the usefulness of the zebrafish embryonic kidney as a model to further understand the function of cilia in kidney health.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5053263PMC
http://dx.doi.org/10.1002/dvg.22957DOI Listing

Publication Analysis

Top Keywords

cilia
10
kidney
9
kidney development
8
role cilia
8
kidney disease
8
cilia kidney
8
antennas organ
4
organ morphogenesis
4
morphogenesis roles
4
roles cilia
4

Similar Publications

Prominosomes - a particular class of extracellular vesicles containing prominin-1/CD133?

J Nanobiotechnology

January 2025

Biotechnology Center (BIOTEC) and Center for Molecular and Cellular Bioengineering, Technische Universität Dresden, Tatzberg 47-49, 01307, Dresden, Germany.

Extracellular membrane vesicles (EVs) offer promising values in various medical fields, e.g., as biomarkers in liquid biopsies or as native (or bioengineered) biological nanocarriers in tissue engineering, regenerative medicine and cancer therapy.

View Article and Find Full Text PDF

Outer dynein arms (ODAs) are essential for ciliary motility and are preassembled in the cytoplasm before trafficking into cilia by intraflagellar transport (IFT). ODA16 is a key adaptor protein that links ODAs to the IFT machinery via a direct interaction with the IFT46 protein. However, the molecular mechanisms regulating the assembly, transport, and release of ODAs remain poorly understood.

View Article and Find Full Text PDF

Ciliopathy-associated missense mutations in IFT140 are tolerated by the inherent resilience of the IFT machinery.

Mol Cell Proteomics

January 2025

Institute for Ophthalmic Research, Center for Ophthalmology, University of Tübingen, Elfriede-Aulhorn-Strasse 7, 72076 Tübingen, Germany. Electronic address:

Genotype-phenotype correlations of rare diseases are complicated by low patient number, high phenotype variability and compound heterozygosity. Mutations may cause instability of single proteins, and affect protein complex formation or overall robustness of a specific process in a given cell. Ciliopathies offer an interesting case for studying genotype-phenotype correlations as they have a spectrum of severity and include diverse phenotypes depending on different mutations in the same protein.

View Article and Find Full Text PDF
Article Synopsis
  • Primary ciliary dyskinesia (PCD) is a rare genetic disorder linked to chronic respiratory issues, infertility, and problems with body asymmetry, primarily caused by mutations in the CCDC39 and CCDC40 genes.
  • Researchers used advanced techniques to investigate how these genetic variants impact cellular functions beyond just causing cilia to stop moving.
  • They discovered that the absence of CCDC39/CCDC40 creates a significant loss of over 90 ciliary structural proteins, leading to cilia dysfunction and other cellular issues, suggesting that gene therapy could potentially offer a new treatment strategy for PCD.
View Article and Find Full Text PDF

A hyperelastic torque-reversal mechanism for soft joints with compression-responsive transient bistability.

Sci Robot

January 2025

Biorobotics Laboratory, Soft Robotics Research Center, Institute of Advanced Machines and Design, Department of Mechanical Engineering, Institute of Engineering, Seoul National University, Seoul, Republic of Korea.

Snap-through, a rapid transition of a system from an equilibrium state to a nonadjacent equilibrium state, is a valuable design element of soft devices for converting a monolithic stimulus into systematic responses with impulsive motions. A common way to benefit from snap-through is to embody it within structures and materials, such as bistable structures. Torque-reversal mechanisms discovered in nature, which harness snap-through instability via muscular forces, may have comparative advantages.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!