Navigation Links
JCI online early table of contents: Oct. 10, 2011
Date:10/10/2011

onal capacity and reduced life span. It is an inherited condition caused by a mutant HTT gene. Although this has been known for many years, the functions of the normal Htt protein and the mechanisms by which the mutant protein generated from the mutant HTT gene causes disease are not well understood. A team of researchers led by Frdric Saudou, at the Institut Curie, France, has now uncovered a new function for normal Htt protein and determined that this function is disrupted in a mouse model of Huntington disease and in patients with the disorder.

Detailed analysis by Saudou and colleagues determined that normal Htt protein regulates the formation of cellular structures known as cilia and that cilia were longer and disorganized in the mouse model of Huntington disease and patients. They therefore suggest that abnormal cilia could be a cause of some of the symptoms of Huntington disease. However, they also caution that further studies are needed to prove this. This point is also made in an accompanying commentary by Scott Zeitlin and Jeh-Ping Liu, at the University of Virginia, Charlottesville, who go on to note that determining this is critical to discerning whether therapeutic strategies designed to normalize ciliary function could ameliorate the symptoms of Huntington disease.

TITLE: Ciliogenesis is regulated by a huntingtin-HAP1-PCM1 pathway and is altered in Huntington disease

AUTHOR CONTACT:
Frdric Saudou
Institut Curie, CNRS UMR 3306, INSERM U1005, Orsay, France.
Phone: 33.1.69.86.30.24; Fax: 33.1.69.86.30.17; E-mail: Frederic.Saudou@curie.fr.

View this article at: http://www.jci.org/articles/view/57552?key=b408131883a0d00ac557

ACCOMPANYING COMMENTARY
TITLE: The long and the short of aberrant ciliogenesis in Huntington disease

AUTHOR CONTACT:
Scott O. Zeitlin
'/>"/>

Contact: Karen Honey
press_releases@the-jci.org
734-546-5242
Journal of Clinical Investigation
Source:Eurekalert

Page: 1 2 3 4 5 6

Related biology news :

1. TGen spin-off MedTrust Online enables cancer doctors to quickly obtain best-available information
2. Personalized medicine: Innovative online journal leads the way
3. Latin American Science Initiative Puts Tropical Rainforest Diversity Online
4. Smithsonian puts tropical Eastern-Pacific shore fishes online
5. Smithsonian puts tropical eastern Pacific shore fishes online
6. Powerful online tool for protein analysis provided pro bono by Stanford geneticist
7. New online report on massive jellyfish swarms released
8. JCI online early table of contents: Jan. 19, 2009
9. Online collaboration identifies bacteria
10. Free online toolkit provides standard measures for genome and population studies
11. K-State plant pathologists develop online teaching modules used globally
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:11/11/2014)...  Forensicon, Inc., a Chicago -based ... the promotion of Yaniv Schiff from Senior ... Schiff,s new role as Director, he will lead company ... examiners and provide leadership within the company,s digital forensics ... Schiff joined Forensicon in 2006 ...
(Date:11/6/2014)... team of Florida State University biologists could lead to ... and survive environmental swings such as droughts or floods. ... the journal The Plant Cell , sheds light ... is organized in a cell and how plants regulate ... and others are turned off. , "If you understand ...
(Date:11/5/2014)... career to understanding the Earth,s climate and communicating ... deputy director and regional climatologist for the Western ... Redmond has more than three decades of experience ... data to the general public. , At its ... December 15-19, 2014 the American Geophysical Union (AGU) ...
Breaking Biology News(10 mins):Yaniv Schiff Promoted to Director of Digital Forensics at Forensicon, Inc. 2Maize analysis yields whole new world of genetic science 2Nevada climatologist to be honored at AGU Fall Meeting for lifetime of public outreach 2
... New Haven, Conn.Newly created neurons in adults rely on ... and survival before they can communicate with existing neighboring ... of adult neural stem cells to replace brain cells ... researchers report in The Journal of Neuroscience. In ...
... parents helps them better understand the diseases their children ... offering practical advice for preventing infections in their kids ... Complete Guide to Protecting (without Overprotecting) Your Family from ... inside source on germ defense from antibiotics and ...
... of proteins have finally explained why computer models of ... observations. This work could have vast implications in bioengineering ... relationship between structure and function in these basic building ... published as the cover article of the Oct. 16 ...
Cached Biology News:Handbook helps parents deal with childhood infections 2Speed plays crucial role in breaking protein's H-bonds 2Speed plays crucial role in breaking protein's H-bonds 3
(Date:11/22/2014)... 2014 The “Chiral Chromatography Columns ... Material (Metal, Glass, Plastic), by Application [GC, LC ... Forecast to 2018” analyses and studies the major ... America, Europe, Asia, and Rest of the World. ... figures spread through 135 pages and in-depth TOC ...
(Date:11/21/2014)... November 21, 2014 , ...   Mariano Rodríguez es elegid vicepresidente senior ... KLOX está en marcha para comenzar de forma rápida ... heridas de reciente aprobación en Europa   , ... complace al anunciar los siguientes nombramientos: Todd ...
(Date:11/21/2014)... (PRWEB) November 20, 2014 Author Matthew ... Walk to the Other Side” published as a ... Finalist in the “Fiction: Short Story” category of the ... Keen, President and CEO of USA Book News, said ... and independent publishers, including Simon & Schuster, Penguin, John ...
(Date:11/21/2014)... Fayetteville, Arkansas (PRWEB) November 20, 2014 ... partners, Pinnacle Ozone Solutions, LLC (Pinnacle), and PCI, ... equipment, announced that they have been selected to ... City of Fayetteville, Arkansas Paul R. Noland Wastewater ... feature BlueInGreen’s patented HyDOZ® gas dissolution ...
Breaking Biology Technology:Chiral Chromatography Columns Market worth $87.8 Million by 2018 - New Research Report by MarketsandMarkets 2Chiral Chromatography Columns Market worth $87.8 Million by 2018 - New Research Report by MarketsandMarkets 3Chiral Chromatography Columns Market worth $87.8 Million by 2018 - New Research Report by MarketsandMarkets 4Chiral Chromatography Columns Market worth $87.8 Million by 2018 - New Research Report by MarketsandMarkets 5KLOX Technologies anuncia sus nombramientos ejecutivos 2KLOX Technologies anuncia sus nombramientos ejecutivos 3KLOX Technologies anuncia sus nombramientos ejecutivos 4KLOX Technologies anuncia sus nombramientos ejecutivos 5Mystic Ink 's Short Story Collection “A Short Walk to the Other Side” Honoring Ray Bradbury Praised As Award-Winning Finalist In USA Best Book Awards 2Mystic Ink 's Short Story Collection “A Short Walk to the Other Side” Honoring Ray Bradbury Praised As Award-Winning Finalist In USA Best Book Awards 3Mystic Ink 's Short Story Collection “A Short Walk to the Other Side” Honoring Ray Bradbury Praised As Award-Winning Finalist In USA Best Book Awards 4City of Fayetteville Leads Nation with Innovative Ozone Technology 2City of Fayetteville Leads Nation with Innovative Ozone Technology 3City of Fayetteville Leads Nation with Innovative Ozone Technology 4
... Mass., June 2 Cynosure, Inc. (Nasdaq: ... of light-based aesthetic treatment systems, today announced that President and Chief Executive ... present at the following investor conferences in New York City this month: ... Gabelli & Company Healthcare Symposium , Tuesday, ...
... ... ClearTrial will lead panel of adaptive trial experts offering practical guidelines ... become more prevalent, their operational planning challenges often remain overlooked in ... on the initial trial design, ranging from understanding the potential for ...
... Micromet, Inc. (Nasdaq: MITI ), a biopharmaceutical company developing ... diseases, today announced that it will host a webcast on June ... and 6th at the 14th Congress of the European Hematology Association. ... www.micromet-inc.com . , , Forum: ...
Cached Biology Technology:Cynosure Announces Presentations at Upcoming Investor Conferences 2ClearTrial to Chair Session at DIA 45th Annual Meeting on "Effective Operational Planning for Adaptive Clinical Trials" 2ClearTrial to Chair Session at DIA 45th Annual Meeting on "Effective Operational Planning for Adaptive Clinical Trials" 3Micromet to Host Conference Call and Webcast to Discuss Blinatumomab Data Presented at EHA on June 8, 2009 2Micromet to Host Conference Call and Webcast to Discuss Blinatumomab Data Presented at EHA on June 8, 2009 3Micromet to Host Conference Call and Webcast to Discuss Blinatumomab Data Presented at EHA on June 8, 2009 4
... normal tissues this enzyme exists predominantly as ... uric acid, a critical plasma antioxidant. ... the oxygen radical producing XO by oxidation ... limited proteolytic cleavage of the amino-terminus. ...
... bridges the gap between the 500 l ... combines the speed of the classic Vivaspin ... area for superior recoveries from very dilute ... PES, Cellulose Triacetate membranes, Vivaspin 2 offers ...
Goat polyclonal to Lipocalin 2...
... two unrelated proteins both of which function ... cycle in a p53-dependent manner. p14^ARF^ binds ... of mdm2 proteinrequired for mdm2 modification and ... interaction is mediated by the exon 1Beta-encoded ...
Biology Products: