Navigation Links
Blame the 'chaperone'
Date:1/7/2011

A Jackson Laboratory research team led by Professor Patsy Nishina, Ph.D., has identified a mutation in a gene that's essential for correct protein-processing in cells. Defects in protein folding are associated with a variety of abnormalities and diseases.

Cells don't come prefabricated, with pieces plunked down and tacked together like modular homes offloaded from trucks. The structural proteins that give cells shape, tubulin and actin (think beams and girders), are themselves subject to essential processing before they become part of the assembly. Proteins must be folded in very specific ways to function properly, and depend on "chaperone" proteins to help them in the process. Defects in the chaperone proteins lead to disruption of the folding process, which adversely affects cellular development and growth.

The chaperones themselves, such as prefoldin, are quite complicated. Prefoldin, which stabilizes brand new proteins, has six subunits assembled into a structure that looks like a jellyfish. Nishina and colleagues identified a mutation in one of the subunits of prefoldin, Pfdn5. In mice, it causes photoreceptor degeneration in the eye, central nervous system abnormalities and male infertility. Although Pfdn5 is widely expressed in other tissues, its disruption appears to significantly affect only these cells. Defects in other subunits disrupt development in other tissues, indicating that each prefoldin subunit is important for the processing of different proteins in different tissues.

The complex protein transport and folding process is only beginning to be fully studied in mammals. Given that disruptions in the process contribute to a variety of human disease mechanisms, the Pdfn5 mutation will be a valuable tool for further research.


'/>"/>

Contact: Joyce Peterson
joyce.peterson@jax.org
207-288-6058
Jackson Laboratory
Source:Eurekalert

Page: 1

Related biology news :

1. New report: Dont blame the pill for estrogen in drinking water
2. Blame the environment: Why vaccines may be ineffective for some people
3. Lacking Finance Education to Blame for Rising Insolvency?
4. Dont blame cities for climate change, see them as solutions
5. Dynamics of chaperone protein critical in rescuing brains of Alzheimers mice from neuron damage
6. Breakthrough: With a chaperone, copper breaks through
7. Molecular chaperone keeps bacterial proteins from slow-dancing to destruction
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/2/2016)... 2, 2016   The Weather Company , an IBM ... an industry-first capability in which consumers will be able to ... ask questions via voice or text and receive relevant information ... Marketers have long sought an advertising solution that ... be personal, relevant and valuable; and can scale across millions ...
(Date:6/1/2016)... 1, 2016 Favorable Government Initiatives ... and Criminal Identification to Boost Global Biometrics System Market ... TechSci Research report, " Global Biometrics Market By ... and Opportunities, 2011 - 2021", the global biometrics market ... on account of growing security concerns across various end ...
(Date:5/20/2016)... -- VoiceIt is excited to announce its new marketing ... working together, VoiceIt and VoicePass will offer an ... slightly different approaches to voice biometrics, collaboration between ... Both companies ... "This marketing and technology partnership allows VoiceIt ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... 2016 , ... Charm Sciences, Inc. is pleased to announce ... Research Institute approval 061601. , “This is another AOAC-RI approval of the Peel ... President of Regulatory and Industrial Affairs. “The Peel Plate methods perform comparably to ...
(Date:6/23/2016)... ... June 23, 2016 , ... STACS DNA Inc., the sample ... the Arkansas State Crime Laboratory, has joined STACS DNA as a Field Application Specialist. ... said Jocelyn Tremblay, President and COO of STACS DNA. “In further expanding our capacity ...
(Date:6/23/2016)... NEW YORK , June 23, 2016 ... the trading session at 4,833.32, down 0.22%; the Dow Jones ... the S&P 500 closed at 2,085.45, down 0.17%. Stock-Callers.com has ... INFI ), Nektar Therapeutics (NASDAQ: NKTR ... BIND Therapeutics Inc. (NASDAQ: BIND ). Learn more ...
(Date:6/23/2016)... PUNE, India , June 23, 2016 /PRNewswire/ ... culture media market research report to its pharmaceuticals ... company profiles, product details and much more. ... market spread across 151 pages, profiling 15 companies ... now available at http://www.reportsnreports.com/reports/601420-global-cell-culture-media-industry-2016-market-research-report.html . ...
Breaking Biology Technology: