Featured Research

from universities, journals, and other organizations

Bardet-Biedl syndrome proteins shown to run an export business that protects cilia

Date:
January 4, 2010
Source:
Rockefeller University Press
Summary:
A protein complex mutated in human disease removes excess signaling molecules to prevent them from damaging cilia, say researchers.

A protein complex mutated in human disease removes excess signaling molecules to prevent them from damaging cilia, say researchers from UMass Medical School. The study will be published in the December 28 issue of the Journal of Cell Biology.

Defective cilia cause a range of diseases including Bardet-Biedl syndrome (BBS), a rare, multi-tissue disorder linked to mutations in 12 different proteins. Seven of these form a complex called the BBSome, but the function of this protein assembly in cilia and flagella is unclear. In worms, the complex glues together the intraflagellar transport (IFT) machinery that assembles and maintains cilia by hauling cargo back and forth along the organelle's microtubules. But most mammalian cell types can still form cilia in the absence of BBS proteins, suggesting that the BBSome isn't essential for IFT.

Lechtreck et al. turned to the green alga Chlamydomonas, and found that BBS proteins were only present on a subset of IFT particles in each of the alga's two flagella. Strains lacking components of the BBSome showed normal rates of IFT and proper flagellar structure, but couldn't steer away from bright light like wild-type cells could. Mutant flagella accumulated several signaling-related proteins, which the researchers think may disrupt the alga's response to light.

The researchers speculate that a similar buildup of disruptive proteins causes cilia dysfunction in BBS patients; the BBSome may remove excess signaling proteins from flagella by linking them to a subset of IFT particles undergoing retrograde transport out of the cilia. Author Karl Lechtreck says that the next step is to fluorescently tag the signaling proteins and compare their movements to BBS and IFT proteins.


Story Source:

The above story is based on materials provided by Rockefeller University Press. Note: Materials may be edited for content and length.


Journal Reference:

  1. Karl-Ferdinand Lechtreck, Eric C. Johnson, Tsuyoshi Sakai, Deborah Cochran, Bryan A. Ballif, John Rush, Gregory J. Pazour, Mitsuo Ikebe, and George B. Witman. The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagella. The Journal of Cell Biology, 2009; 187 (7): 1117 DOI: 10.1083/jcb.200909183

Cite This Page:

Rockefeller University Press. "Bardet-Biedl syndrome proteins shown to run an export business that protects cilia." ScienceDaily. ScienceDaily, 4 January 2010. <www.sciencedaily.com/releases/2009/12/091228105909.htm>.
Rockefeller University Press. (2010, January 4). Bardet-Biedl syndrome proteins shown to run an export business that protects cilia. ScienceDaily. Retrieved July 30, 2014 from www.sciencedaily.com/releases/2009/12/091228105909.htm
Rockefeller University Press. "Bardet-Biedl syndrome proteins shown to run an export business that protects cilia." ScienceDaily. www.sciencedaily.com/releases/2009/12/091228105909.htm (accessed July 30, 2014).

Share This




More Health & Medicine News

Wednesday, July 30, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Concern Grows Over Worsening Ebola Crisis

Concern Grows Over Worsening Ebola Crisis

AFP (July 30, 2014) Pan-African airline ASKY has suspended all flights to and from the capitals of Liberia and Sierra Leone amid the worsening Ebola health crisis, which has so far caused 672 deaths in Guinea, Liberia and Sierra Leone. Duration: 00:43 Video provided by AFP
Powered by NewsLook.com
Generics Eat Into Pfizer's Sales

Generics Eat Into Pfizer's Sales

Reuters - Business Video Online (July 29, 2014) Pfizer, the world's largest drug maker, cut full-year revenue forecasts because generics could cut into sales of its anti-arthritis drug, Celebrex. Fred Katayama reports. Video provided by Reuters
Powered by NewsLook.com
Nigeria Ups Ebola Stakes on 1st Death

Nigeria Ups Ebola Stakes on 1st Death

Reuters - Business Video Online (July 29, 2014) Nigerian authorities have shut and quarantined a Lagos hospital where a Liberian man died of the Ebola virus, the first recorded case of the highly-infectious disease in Africa's most populous economy. David Pollard reports Video provided by Reuters
Powered by NewsLook.com
Running 5 Minutes A Day Might Add Years To Your Life

Running 5 Minutes A Day Might Add Years To Your Life

Newsy (July 29, 2014) According to a new study, just five minutes of running or jogging a day could add years to your life. Video provided by Newsy
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:
from the past week

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