Featured Research

from universities, journals, and other organizations

Critical Protein Complex In Formation Of Cell Cilia Identified

Date:
August 20, 2008
Source:
NYU Langone Medical Center / New York University School of Medicine
Summary:
Scientists have identified a protein complex that regulates the formation of cilia, which are found on virtually all mature human cells and are essential to normal cell function.

An international team led by NYU Cancer Institute have identified a protein complex that regulates the formation of cilia, which are found on virtually all mature human cells and are essential to normal cell function.

Related Articles


The new report describes how three proteins work together to regulate the formation of primary cilia. The study led by Brian Dynlacht, Ph.D., professor of pathology and director of NYU Cancer Institute Genomics Facility, investigates these antenna-like structures, once thought to be vestigial remnants of cell evolution, which have recently emerged as a focal point of research in developmental cell biology.

"We are trying to understand the regulation of processes that are fundamental to normal cell development and health in humans," said William Y. Tsang, Ph.D., of the NYU School of Medicine and Cancer Institute, and first author of the paper. "Defective cilia are implicated in a wide range of serious illnesses such as polycystic kidney disease, retinal degeneration, and neurological disorders. Inappropriate activation of signaling molecules that normally reside at the primary cilium, may lead to certain cancers."

At the center of the process lies the protein CEP290, which normally promotes primary cilia formation in mature cells. Dr. Tsang and his colleagues discovered that a second protein, CP110, normally suppresses the function of CEP290 until cells are fully mature. At that point, CP110 is destroyed, freeing CEP290 to interact with a third protein, Rab8a, to promote cilia formation on the surface of the mature cell.

The team's findings may help to identify potential targets for future drug design.

"Ciliogenesis is a fundamental process. These structures are found in almost every type of human cell you can imagine," Dr. Tsang said. "If we can ever design drugs that will restore the formation and function of cilia even in the presence of CEP290 mutations, then that would be one way to cure the defects that lead to ciliary diseases."

Research so far has been using in vitro human cell lines. However, team members from the University of Michigan and National Eye Institute have developed a mouse model with a CEP290 mutation implicated in retinal degeneration, and the NYU group is planning a study of human CEP290 mutations to see if they can correlate genotypes to their expression in specific ciliary diseases.

The authors of this study are NYU Cancer Institute scientists William Y. Tsang and Brian David Dynlacht; Carine Bossard (Centre for Genomic Regulation, Barcelona); Hemant Khanna (Department of Ophthalmology and Visual Sciences, University of Michigan); Johan Perδnen (Institute of Biotechnology, Program in Cellular Biotechnology, University of Helsinki), Anand Swaroop (Department of Ophthalmology and Visual Sciences, University of Michigan / National Eye Institute, Neurobiology Neurodegeneration & Repair Laboratory, Bethesda, MD); and Vivek Malhotra (Centre for Genomic Regulation, Barcelona).


Story Source:

The above story is based on materials provided by NYU Langone Medical Center / New York University School of Medicine. Note: Materials may be edited for content and length.


Journal Reference:

  1. Tsang et al. CP110 Suppresses Primary Cilia Formation through Its Interaction with CEP290, a Protein Deficient in Human Ciliary Disease. Developmental Cell, 2008; 15 (2): 187 DOI: 10.1016/j.devcel.2008.07.004

Cite This Page:

NYU Langone Medical Center / New York University School of Medicine. "Critical Protein Complex In Formation Of Cell Cilia Identified." ScienceDaily. ScienceDaily, 20 August 2008. <www.sciencedaily.com/releases/2008/08/080819160247.htm>.
NYU Langone Medical Center / New York University School of Medicine. (2008, August 20). Critical Protein Complex In Formation Of Cell Cilia Identified. ScienceDaily. Retrieved December 20, 2014 from www.sciencedaily.com/releases/2008/08/080819160247.htm
NYU Langone Medical Center / New York University School of Medicine. "Critical Protein Complex In Formation Of Cell Cilia Identified." ScienceDaily. www.sciencedaily.com/releases/2008/08/080819160247.htm (accessed December 20, 2014).

Share This


More From ScienceDaily



More Plants & Animals News

Saturday, December 20, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Researchers Test Colombian Village With High Alzheimer's Rates

Researchers Test Colombian Village With High Alzheimer's Rates

AFP (Dec. 19, 2014) — In Yarumal, a village in N. Colombia, Alzheimer's has ravaged a disproportionately large number of families. A genetic "curse" that may pave the way for research on how to treat the disease that claims a new victim every four seconds. Duration: 02:42 Video provided by AFP
Powered by NewsLook.com
Monarch Butterflies Descend Upon Mexican Forest During Annual Migration

Monarch Butterflies Descend Upon Mexican Forest During Annual Migration

Reuters - Light News Video Online (Dec. 19, 2014) — Millions of monarch butterflies begin to descend onto Mexico as part of their annual migration south. Rough Cut (no reporter narration) Video provided by Reuters
Powered by NewsLook.com
Birds Might Be Better Meteorologists Than Us

Birds Might Be Better Meteorologists Than Us

Newsy (Dec. 19, 2014) — A new study suggests a certain type of bird was able to sense a tornado outbreak that moved through the U.S. a day before it hit. Video provided by Newsy
Powered by NewsLook.com
Navy Unveils Robot Fish

Navy Unveils Robot Fish

Reuters - Light News Video Online (Dec. 18, 2014) — The U.S. Navy unveils an underwater device that mimics the movement of a fish. Tara Cleary reports. Video provided by Reuters
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Plants & Animals

Earth & Climate

Fossils & Ruins

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins