Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Scientists Identify Critical Protein Complex in Formation of Cell Cilia

21.08.2008
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.

The new report, published this week by Developmental Cell and selected as the featured publication of the open-access online edition, 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 NYU 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 Peränen (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).

The complete article is available at: http://www.developmentalcell.com/content/article/fulltext?uid=PIIS1534580708002839

Jennifer Berman | Newswise Science News
Further information:
http://www.nyumc.org

More articles from Life Sciences:

nachricht Seeing on the Quick: New Insights into Active Vision in the Brain
15.08.2018 | Eberhard Karls Universität Tübingen

nachricht New Approach to Treating Chronic Itch
15.08.2018 | Universität Zürich

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Unraveling the nature of 'whistlers' from space in the lab

A new study sheds light on how ultralow frequency radio waves and plasmas interact

Scientists at the University of California, Los Angeles present new research on a curious cosmic phenomenon known as "whistlers" -- very low frequency packets...

Im Focus: New interactive machine learning tool makes car designs more aerodynamic

Scientists develop first tool to use machine learning methods to compute flow around interactively designable 3D objects. Tool will be presented at this year’s prestigious SIGGRAPH conference.

When engineers or designers want to test the aerodynamic properties of the newly designed shape of a car, airplane, or other object, they would normally model...

Im Focus: Robots as 'pump attendants': TU Graz develops robot-controlled rapid charging system for e-vehicles

Researchers from TU Graz and their industry partners have unveiled a world first: the prototype of a robot-controlled, high-speed combined charging system (CCS) for electric vehicles that enables series charging of cars in various parking positions.

Global demand for electric vehicles is forecast to rise sharply: by 2025, the number of new vehicle registrations is expected to reach 25 million per year....

Im Focus: The “TRiC” to folding actin

Proteins must be folded correctly to fulfill their molecular functions in cells. Molecular assistants called chaperones help proteins exploit their inbuilt folding potential and reach the correct three-dimensional structure. Researchers at the Max Planck Institute of Biochemistry (MPIB) have demonstrated that actin, the most abundant protein in higher developed cells, does not have the inbuilt potential to fold and instead requires special assistance to fold into its active state. The chaperone TRiC uses a previously undescribed mechanism to perform actin folding. The study was recently published in the journal Cell.

Actin is the most abundant protein in highly developed cells and has diverse functions in processes like cell stabilization, cell division and muscle...

Im Focus: Lining up surprising behaviors of superconductor with one of the world's strongest magnets

Scientists have discovered that the electrical resistance of a copper-oxide compound depends on the magnetic field in a very unusual way -- a finding that could help direct the search for materials that can perfectly conduct electricity at room temperatur

What happens when really powerful magnets--capable of producing magnetic fields nearly two million times stronger than Earth's--are applied to materials that...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Within reach of the Universe

08.08.2018 | Event News

A journey through the history of microscopy – new exhibition opens at the MDC

27.07.2018 | Event News

2018 Work Research Conference

25.07.2018 | Event News

 
Latest News

Unraveling the nature of 'whistlers' from space in the lab

15.08.2018 | Physics and Astronomy

Diving robots find Antarctic winter seas exhale surprising amounts of carbon dioxide

15.08.2018 | Earth Sciences

Early opaque universe linked to galaxy scarcity

15.08.2018 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>