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

0

GOOD

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:5/23/2013)... with autologous cord blood. Following a cardiac arrest with ... been in a persistent vegetative state with minimal ... the cord blood containing stem cells, the symptoms improved ... speak simple sentences and to move. "Our findings, along ... doubts about the effectiveness of the new therapy", says ...
(Date:5/22/2013)... NEW YORK CITY, May 22, 2013 U.S. Forest ... multi-disciplinary team that, with funding from the TKF Foundation, ... spaces help communities and individuals recover from tragedy. , ... is one of six projects selected for grant funding. ... spaces and sacred spaces in recovery and resiliency, the ...
(Date:5/22/2013)... 22, 2013)The omega 3 fatty acids in fish oil ... much so that the American Heart Association currently recommends ... particularly fatty varieties rich in omega 3s. However, the ... In a new study, scientists led by Jason R. ... phenomenon by providing evidence that fish oil might specifically ...
Breaking Biology News(10 mins):First successful treatment of pediatric cerebral palsy with autologous cord blood 2'Landscapes of Resilience' to study how people use nature as a source of recovery 2Fish oil may help the heart beat mental stress 2
... and US scientists has found there has been a massive ... the coast of Antarctica.,Comparing detailed measurements taken during the Australian ... data dating back to 1970, scientists estimate there has been ... volume of Antarctic Bottom Water, the cold dense water that ...
... IMPACT World+, the first worldwide regionalized life cycle impact ... during the 6th SETAC World Congress / SETAC Europe ... this innovative undertaking spearheaded by a team of leading ... production and consumption of goods and services generate significant ...
... able to target and destroy triple negative breast cancer, ... access journal Breast Cancer Research . Researchers from ... was able to destroy breast cancer cells and reduce ... cancers are found at diagnosis to be triple negative. ...
Cached Biology News:Latest Southern Ocean research shows continuing deep ocean change 2Latest Southern Ocean research shows continuing deep ocean change 3Launch of IMPACT World+ 2Blocking DNA: HDAC inhibitor targets triple negative breast cancer 2
(Date:5/23/2013)... Can economic incentives such as gift cards, T-shirts, ... public to increase their donations of blood? , A ... School Assistant Professor Mario Macis says the answer ... of recent research that supports their argument, the three ... Science that the World Health Organization and national blood ...
(Date:5/23/2013)...  Rahal Letterman Lanigan Racing (RLL) and StemCyte, Inc., ... banking and therapeutics, announced today that StemCyte has become ... Indy car driven by James Jakes ... IndyCar 2013 season.   StemCyte, Inc. and Rahal ... of umbilical cord blood banking and its educational initiative ...
(Date:5/23/2013)... LAGUNA HILLS, Calif. , May 23, 2013 /PRNewswire-USNewswire/ ... CA recently became the first hospital on ... site robotic technology. This new surgical option provides women ... Marc Winter , ... gynecologist and surgeon and medical director of minimally ...
(Date:5/23/2013)... Z Trim Holdings, Inc. (OTC Markets: ZTHO), ... of industries, announced that Chef Erin Ryan ... year,s National Restaurant Association ("NRA") NRA Show, which was ... Research Chefs Association.  Chef Ryan spoke on the subject ... and the Ugly," joining four other prestigious panelists including: ...
Breaking Biology Technology:Offering Economic Incentives to Attract Blood Donations Should Be Encouraged, Research Team writes in Science 2Offering Economic Incentives to Attract Blood Donations Should Be Encouraged, Research Team writes in Science 3StemCyte, Inc. Joins Rahal Letterman Lanigan Racing As Associate Sponsor Of No. 16 IndyCar Program For James Jakes Beginning With The 97th Indianapolis 500 2StemCyte, Inc. Joins Rahal Letterman Lanigan Racing As Associate Sponsor Of No. 16 IndyCar Program For James Jakes Beginning With The 97th Indianapolis 500 3Saddleback Memorial Surgeon First on the West Coast to Perform Single-Site Robotic Hysterectomy 2Saddleback Memorial Surgeon First on the West Coast to Perform Single-Site Robotic Hysterectomy 3Z Trim's Chef Erin Ryan sits on Expert Panel at NRA Show 2Z Trim's Chef Erin Ryan sits on Expert Panel at NRA Show 3
... Israel, Nov. 29 Protalix,BioTherapeutics, Inc. (Amex: PLX ... the close of business on December 24, 2007, as ... and to vote at, the,Company,s 2007 Annual Meeting of ... Meeting of Shareholders will be held on,January 31, 2008, ...
... the treatment ... disease ... Symbol: RVX, CALGARY, Nov. 29 /PRNewswire-FirstCall/ - Resverlogix Corp.,("Resverlogix") (TSX:RVX) is pleased ... of the very prestigious,Technology Pioneer Award in recognition of its NexVas(TM) Plaque Removal,program. ...
... Jerusalem Global Ventures today,announced that it will soon ... fund. The Fund will invest in private Israeli related,technology ... three years and,have a proven business model. First closing ... who founded Jerusalem Global in 1994, notes that,"Unlike the ...
Cached Biology Technology:Protalix BioTherapeutics Sets Record Date for Annual Meeting of Shareholders 2Protalix BioTherapeutics Sets Record Date for Annual Meeting of Shareholders 3Resverlogix Honoured with World Economic Forum Technology Pioneer Award 2Resverlogix Honoured with World Economic Forum Technology Pioneer Award 3Resverlogix Honoured with World Economic Forum Technology Pioneer Award 4Resverlogix Honoured with World Economic Forum Technology Pioneer Award 5Jerusalem Global Ventures Announces $200 Million Growth Equity Fund 2
... CHEMICON APO-BRDU Kit is a ... breaks and total cellular DNA to detect ... kit contains the instructions and reagents required ... and negative control cells for assessing reagent ...
... with temperature probe, 100-120 and 220-240 V, supports ... and 400 W. The temperature probe is used ... degrees C during electrophoresis. Built-in infrared capability allows ... are provided. Dimensions are 27.5 x 34 x ...
The Trans-Blot Plus gel and cassette assembly tray is used to hold the Trans-Blot Plus gel holder cassette, buffer, and other materials for the gel and membrane sandwich during their assembly....
Criterion staining and blotting trays are plastic trays designed for staining one or two Criterion gels or performing western blot detection. The trays have a working capacity of up to 500 ml. Suppli...
Biology Products: