Corporate Banner
Satellite Banner
Technology
Networks
Scientific Communities
 
Become a Member | Sign in
Home>News>This Article
  News
Return

Surprising Mechanism Discovered in Polycystic Kidney Disease

Published: Monday, July 29, 2013
Last Updated: Monday, July 29, 2013
Bookmark and Share
A study has uncovered a new and unexpected molecular mechanism in the development of polycystic kidney disease, or PKD.

PKD is a life-threatening genetic disorder that causes multiple cysts to form on the kidneys — enlarging them, cutting off proper urine flow, and causing kidney failure in half of affected people by age 60. It affects more than 12 million people worldwide.

Cilia are the hair-like structures on the surface of many human cells that can either move things along – dirt out of the lungs, or an egg from the ovary to the uterus – or sense the environment, such as vision in the retina or smell in the nose. Recent research has implicated defects in the sensory cilia — often caused by genetic mutations — in many human diseases, including cancer, cardiac disease, blindness, and kidney disease. In the kidney, disruption of sensory cilia cause kidney cysts.

The polycystin-1 and -2 (also known as PC1 and PC2) proteins are key players in the normal functioning of the kidneys. Earlier research has shown that when they are lost or mutated, cysts grow in the kidneys and cause almost all cases of PKD in humans.

Working in mice, the Yale team found that cysts grew when the cilia were intact but lacked polycystin — but, surprisingly, cysts stopped growing despite the absence of polycystins when the cilia were disrupted or eliminated.

The activity of this pathway, and the timing of the loss of polycystin proteins and the cilia, determined the severity of both early- and adult-onset PKD, the researchers found.

“None of the other pathways discovered so far have proven as universal as the cilia dependent pathway in explaining polycystic kidney disease,” said corresponding author Dr. Stefan Somlo, professor of internal medicine (nephrology) and genetics at Yale School of Medicine. “We found to our surprise that elimination of cilia suppresses cyst growth in all of the genetic models of human PKD.”

Somlo believes that his team’s research could lead to discovery of new targets for therapies to inhibit this cilia-dependent pathway of PKD, and slow cyst growth.

Other authors are Ming Ma and Xin Tian of Yale, Peter Igarashi of the University of Texas Southwestern School of Medicine, and Gregory Pazour of the University of Massachusetts Medical School.


Further Information
Access to this exclusive content is for Technology Networks Premium members only.

Join Technology Networks Premium for free access to:

  • Exclusive articles
  • Presentations from international conferences
  • Over 2,500+ scientific posters on ePosters
  • More Than 3,800+ scientific videos on LabTube
  • 35 community eNewsletters


Sign In



Forgotten your details? Click Here
If you are not a member you can join here

*Please note: By logging into TechnologyNetworks.com you agree to accept the use of cookies. To find out more about the cookies we use and how to delete them, see our privacy policy.

Related Content

Novel Technique for Kidney Research Developed
To better understand how the treatment leads to kidney damage, and possibly prevent it, a team of researchers at Yale School of Medicine developed a new 3D-imaging technique to peer deep into these vital organs.
Thursday, August 27, 2015
Shedding Light On Century-Old Biochemical Mystery
Yale scientists have used magnetic resonance measurements to show how glucose is metabolized in yeast to answer the puzzle of the “Warburg Effect.”
Thursday, August 20, 2015
Gene Testing Now Allows Precision Medicine for Thoracic Aneurysms
Researchers at the Aortic Institute at Yale have tested the genomes of more than 100 patients with thoracic aortic aneurysms, a potentially lethal condition, and provided genetically personalized care.
Monday, July 20, 2015
Creating More Potent Vaccines
Yale researchers uncovered a new role for a type of immune cell, known as regulatory T cells, in promoting long-term immunity.
Wednesday, July 08, 2015
Yale Team finds why BRCA Gene Resists Cancer Treatment
The University researchers have discovered why a key molecular assistant is crucial to the function of the BRCA2 gene.
Tuesday, July 07, 2015
New Type of Drug Can Target All Disease-causing Proteins
Current drugs block the actions of only about a quarter of known disease-causing proteins, but Yale University researchers have developed a technology capable of not just inhibiting, but destroying every protein it targets.
Monday, June 15, 2015
After a Sip of Milkshake, Genes and Brain Activity Predict Weight Gain
The new study published in The Journal Neuroscience.
Thursday, May 21, 2015
Researchers Solve Multiple Sclerosis Puzzle
Yale study shows the role that T cells play in MS.
Monday, May 18, 2015
Gene Editing Corrects Mutation In Cystic Fibrosis
Yale researchers successfully corrected the most common mutation in the gene that causes cystic fibrosis, a lethal genetic disorder.
Monday, April 27, 2015
New Tool To Explore Mysteries Of The Immune System
Yale scientists use CyTOF to study a range of conditions.
Monday, April 20, 2015
A Faster, Less Expensive Way To Analyze Gene Activity
Yale researchers have devised a method that could reduce the time and cost of analyzing gene activity.
Tuesday, March 03, 2015
Li Ka Shing Foundation Renews Support for Yale Stem Cell Center
New generous grant of $1.86 million from LKSF to support education and healthcare initiatives.
Saturday, February 28, 2015
Single-Cell, 42-plexed Protein Analysis Achieved with a New Microchip Technology
A novel microdevice capable of detecting 42 unique immune effector proteins has been developed.
Tuesday, February 17, 2015
Yale Team Identifies Key Process In Brain Development
miR-107 shown to play essential role in regulating normal brain development.
Friday, February 06, 2015
Cold Virus Replicates Better At Cooler Temperatures
Study shows that the immune response to rhinovirus is influenced by temperature.
Tuesday, January 06, 2015
Scientific News
NIH Study Finds Calorie Restriction Lowers Some Risk Factors for Age-Related Diseases
Two-year trial did not produce expected metabolic changes, but influenced other life span markers.
Immunotherapy Agent Benefits Patients with Drug-Resistant Multiple Myeloma in First Human Trial
Daratumumab proved generally safe in patients, even at the highest doses.
Low-level Arsenic Exposure Before Birth Associated with Early Puberty in Female Mice
Study examine whether low-dose arsenic exposure could have similar health outcomes in humans.
Inciting an Immune Attack On Cancer Cells
A new minimally invasive vaccine that combines cancer cells and immune-enhancing factors could be used clinically to launch a destructive attack on tumors.
‘Mutation-Tracking’ Blood Test for Breast Cancer
Scientists have developed a blood test for breast cancer able to identify which patients will suffer a relapse after treatment, months before tumours are visible on hospital scans.
Cellular Contamination Pathway for Heavy Elements Identified
Berkeley Lab scientists find that an iron-binding protein can transport actinides into cells.
Intensity of Desert Storms May Affect Ocean Phytoplankton
MIT study finds phytoplankton are extremely sensitive to changing levels of desert dust.
Common ‘Heart Attack’ Blood Test May Predict Future Hypertension
Small rises in troponin levels may have value as markers for subclinical heart damage and high blood pressure.
LaVision BioTec Reports on the Neuro Research on the Human Brain After Trauma
Company reports on the work of Dr Ali Ertürk from the Institute for Stroke and Dementia Research at LMU Munich.
NIH Study Shows No Benefit of Omega-3 Supplements for Cognitive Decline
Research was published in the Journal of the American Medical Association.
Scroll Up
Scroll Down
Skyscraper Banner

Skyscraper Banner
Go to LabTube
Go to eposters
 
Access to the latest scientific news
Exclusive articles
Upload and share your posters on ePosters
Latest presentations and webinars
View a library of 1,800+ scientific and medical posters
2,500+ scientific and medical posters
A library of 2,500+ scientific videos on LabTube
3,800+ scientific videos
Close
Premium CrownJOIN TECHNOLOGY NETWORKS PREMIUM FREE!