Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Defect of cilia-assembly protein could cause most common genetic cause of kidney failure

01.04.2003


A protein responsible for the assembly of cell cilia – the hair-like projections from cells – may cause polycystic kidney disease, the most common genetic cause of kidney failure, according to a new study at UT Southwestern Medical Center at Dallas.



The study, which will be published online this week and will appear in a future edition of the Proceedings of the National Academy of Sciences, is the first to directly test the role of cilia in polycystic kidney disease. Previous studies have hinted at a possible link, said Dr. Peter Igarashi, chief of nephrology at UT Southwestern and senior author of the study.

"For a long time, renal cilia have been thought to be unimportant organelles," said Igarashi. "This study and others before it have renewed the interest in what cilia are doing normally and also how abnormalities in cilia cause disease."


Polycystic kidney disease (PKD) afflicts about one in every 500 people and causes fluid-filled cysts to accumulate in the kidney, liver and other organs. Formation of the cysts causes progressive renal failure, which requires dialysis or kidney transplantation. No other effective treatment is available.

To test whether stopping cilia formation causes PKD, researchers created knockout mice missing the gene Kif3, specifically in the kidneys. That gene codes for a motor protein that’s critical in cilia formation and maintenance. Researchers created kidney-specific knockouts because cilia are essential for embryonic development.

The knockout mice had normal kidneys at birth, but researchers found that kidney cysts began to develop about five days later and caused renal failure after about three weeks. Dissection by day 35 showed enlarged kidneys with multiple, fluid-filled cysts that had characteristics similar to cysts found in PKD.

"We are trying to understand the mechanism of cyst formation," said Dr. Fangming Lin, assistant professor of pediatrics and lead author of the study. "Once you understand the mechanism we will have the target to prevent or slow the cyst formation.

"That could eventually lead to a treatment of human polycystic disease."

Other UT Southwestern researchers who worked on the study were Dr. Thomas Hiesberger, assistant professor of internal medicine, and Kimberly Cordes, research assistant. Researchers from Yale University School of Medicine and the University of California, San Diego, School of Medicine also contributed to the study, which was funded by the National Institute of Diabetes and Digestive and Kidney Diseases.


###
To automatically receive news releases from UT Southwestern via e-mail, subscribe at http://lists.utsouthwestern.edu/mailman/listinfo/utswnews


Staishy Bostick Siem | EurekAlert!
Further information:
http://www.swmed.edu/

More articles from Health and Medicine:

nachricht One gene closer to regenerative therapy for muscular disorders
01.06.2017 | Cincinnati Children's Hospital Medical Center

nachricht The gut microbiota plays a key role in treatment with classic diabetes medication
01.06.2017 | University of Gothenburg

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Can we see monkeys from space? Emerging technologies to map biodiversity

An international team of scientists has proposed a new multi-disciplinary approach in which an array of new technologies will allow us to map biodiversity and the risks that wildlife is facing at the scale of whole landscapes. The findings are published in Nature Ecology and Evolution. This international research is led by the Kunming Institute of Zoology from China, University of East Anglia, University of Leicester and the Leibniz Institute for Zoo and Wildlife Research.

Using a combination of satellite and ground data, the team proposes that it is now possible to map biodiversity with an accuracy that has not been previously...

Im Focus: Climate satellite: Tracking methane with robust laser technology

Heatwaves in the Arctic, longer periods of vegetation in Europe, severe floods in West Africa – starting in 2021, scientists want to explore the emissions of the greenhouse gas methane with the German-French satellite MERLIN. This is made possible by a new robust laser system of the Fraunhofer Institute for Laser Technology ILT in Aachen, which achieves unprecedented measurement accuracy.

Methane is primarily the result of the decomposition of organic matter. The gas has a 25 times greater warming potential than carbon dioxide, but is not as...

Im Focus: How protons move through a fuel cell

Hydrogen is regarded as the energy source of the future: It is produced with solar power and can be used to generate heat and electricity in fuel cells. Empa researchers have now succeeded in decoding the movement of hydrogen ions in crystals – a key step towards more efficient energy conversion in the hydrogen industry of tomorrow.

As charge carriers, electrons and ions play the leading role in electrochemical energy storage devices and converters such as batteries and fuel cells. Proton...

Im Focus: A unique data centre for cosmological simulations

Scientists from the Excellence Cluster Universe at the Ludwig-Maximilians-Universität Munich have establised "Cosmowebportal", a unique data centre for cosmological simulations located at the Leibniz Supercomputing Centre (LRZ) of the Bavarian Academy of Sciences. The complete results of a series of large hydrodynamical cosmological simulations are available, with data volumes typically exceeding several hundred terabytes. Scientists worldwide can interactively explore these complex simulations via a web interface and directly access the results.

With current telescopes, scientists can observe our Universe’s galaxies and galaxy clusters and their distribution along an invisible cosmic web. From the...

Im Focus: Scientists develop molecular thermometer for contactless measurement using infrared light

Temperature measurements possible even on the smallest scale / Molecular ruby for use in material sciences, biology, and medicine

Chemists at Johannes Gutenberg University Mainz (JGU) in cooperation with researchers of the German Federal Institute for Materials Research and Testing (BAM)...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Plants are networkers

19.06.2017 | Event News

Digital Survival Training for Executives

13.06.2017 | Event News

Global Learning Council Summit 2017

13.06.2017 | Event News

 
Latest News

Quantum thermometer or optical refrigerator?

23.06.2017 | Physics and Astronomy

A 100-year-old physics problem has been solved at EPFL

23.06.2017 | Physics and Astronomy

Equipping form with function

23.06.2017 | Information Technology

VideoLinks
B2B-VideoLinks
More VideoLinks >>>