Navigation Links
Chemical chaperone could open door to treatment of neurological disorder
Date:2/5/2008

>

Niemann-Pick type C disease is a recessive inherited trait that can originate in one of more than 200 different mutations in the NPC1 gene, which lies on chromosome 18. The mutations lead to production of abnormal NPC1 protein. Normally, NPC1 protein plays an essential role in moving cholesterol out of cells, and if it doesn't function, cholesterol and other lipids accumulate.

Most scientists assumed that Niemann-Pick type C mutations produced NPC1 protein that didn't work correctly. So when a routine test in the Ory lab of a mutated NPC1 protein showed that the protein was in fact active in living cells, the researchers did a double take.

"It is unequivocal that the mutation causes disease in human patients," says Ory, also associate professor of cell biology and physiology. "Yet the mutated protein seemed functional when we introduced it into cells."

When they looked for the explanation for this aberration, Ory and colleagues found that a small proportion of the mutant protein actually could do the job of normal NPC1 protein. It turned out that the mutation caused most newly minted NPC1 protein molecules to fold into the wrong shape or to assume their final shape slowly so that the cell's quality control checkpoints rejected them. But some of the mutant protein molecules assumed the correct shape and made it to their proper destination.

That suggested to the team that Niemann-Pick type C disease possibly could be treated with chemicals that assist the mutant proteins produced in patients. Ory refers to these as chemical chaperones and indicates this approach could help the large NPC1 proteins during the process of folding their long, complex chains so that more of the mutant proteins fold properly and pass through the cell's quality control checkpoints.

In collaboration with the National Institutes of Health Chemical Genomics Center, Ory will next screen more than 200,000 compounds to see which ones increase
'/>"/>

Contact: Gwen Ericson
ericsong@wustl.edu
314-286-0141
Washington University School of Medicine
Source:Eurekalert  

Page: 1 2 3

Related medicine news :

1. Chemical culprit in popcorn workers lung identified
2. American Chemical Societys Weekly Presspac -- Sept. 5, 2007
3. Rohm and Haas to Webcast Presentation at Credit Suisse Chemicals Conference
4. Chemical Diversity Initiates International Prostrate Cancer Discovery Partnership
5. Low Doses of Red Wine Chemical May Fight Diabetes
6. National Academies advisory: genes and toxic chemicals
7. Genomic technologies to identify toxic chemicals should be developed
8. Malaria No More and Sumitomo Chemical Help Protect 1.4 Million Kids from Malaria
9. American Chemical Societys Weekly Presspac -- Oct. 17, 2007
10. Researchers study potential health benefits of natural chemicals in muscadine grape seeds
11. Brains Reward Chemical May Help Spur Obesity
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Chemical chaperone could open door to treatment of neurological disorder
(Date:8/21/2014)... (PRWEB) August 21, 2014 ... & Peripheral Vascular Devices Market by ... Drug-Eluting), Catheter (IVUS, OCT), IVC Filter ... to 2018" analyzes and studies the ... in the U.S. and Canada. , ...
(Date:8/21/2014)... 2014 Bedros Keuilian’s Annual Fitness Business Summit ... attended by many people already earning seven-figure incomes. But his ... usually only open to the owners of Keuilian’s own international ... year’s Fit Body Boot Camp World Conference will also be ... fitness boot camp or are struggling with their own ...
(Date:8/21/2014)... (PRWEB) August 21, 2014 mynt welcomes ... brand promoters to join its growing company. Since its ... million in product sales and continues to gain momentum ... , Rob and Tiffanie came into network marketing ... serviced a number of network marketing companies. Tiffanie needed ...
(Date:8/21/2014)... 2014 Truly hungry, or comforting yourself ... the TV, while driving, or at the computer can ... to be more aware of what they eat and ... Mindfulness” presentation. , Registered dietician and community educator ... questions from 12:30 to 2 p.m. Sept. 5 at ...
(Date:8/21/2014)... One of the emerging trends in the market ... This increases the availability and accessibility of these products to ... Ostomy Care Accessories market to grow at a ... the report, the Global Ostomy Care Accessories market is driven ... the major drivers. The number of procedures is increasing owing ...
Breaking Medicine News(10 mins):Health News:North American Interventional Cardiology and Peripheral Vascular Devices Market worth $5.9 Billion by 2018 - Research Report by MarketsandMarkets 2Health News:North American Interventional Cardiology and Peripheral Vascular Devices Market worth $5.9 Billion by 2018 - Research Report by MarketsandMarkets 3Health News:North American Interventional Cardiology and Peripheral Vascular Devices Market worth $5.9 Billion by 2018 - Research Report by MarketsandMarkets 4Health News:North American Interventional Cardiology and Peripheral Vascular Devices Market worth $5.9 Billion by 2018 - Research Report by MarketsandMarkets 5Health News:Fitness Boot Camp Founder Offers Fifty Scholarships to Fit Body Boot Camp World Conference 2Health News:Fitness Boot Camp Founder Offers Fifty Scholarships to Fit Body Boot Camp World Conference 3Health News:Industry Leaders Rob and Tiffanie Moffitt Join mynt 2Health News:HMHP Workshop to Focus on ‘Mindful’ Eating 2Health News:Ostomy Care Accessories Market 2014 – 2018 Global Forecasts and Analysis in New Research Report at Sandlerresearch.org 2Health News:Ostomy Care Accessories Market 2014 – 2018 Global Forecasts and Analysis in New Research Report at Sandlerresearch.org 3Health News:Ostomy Care Accessories Market 2014 – 2018 Global Forecasts and Analysis in New Research Report at Sandlerresearch.org 4
... HealthDay Reporter , TUESDAY, May 29 (HealthDay News) -- ... because they,re working too hard, over-exercising or not taking enough ... unknowingly have a sleep disorder. As many as 18 ... Sleep Foundation. But researchers estimate that as many as 90 ...
... -- Preventive antiretroviral treatment appears to be an effective ... a new study suggests. HIV, the virus that ... contaminated needles. Immediate treatment after HIV exposure can ... found. More recently, several large randomized, controlled trials -- ...
... Grand Rapids, Mich. (May 29, 2012) Researchers from ... on a project to restore neuron function to parts ... successfully transplanting HD-induced pluripotent stem cells into animal models. ... genetically engineered from human somatic cells such as skin, ...
... Professor Adrian Mulholland and Dr Christopher Woods from Bristol,s ... graphics processing units (GPUs) to simulate the molecular processes ... treat the H1N1-2009 strain of influenza commonly known ... May] in Biochemistry , provide new insight that ...
... The family meal is often touted and encouraged for ... study questions the nature of this association, finding that the ... once a number of factors are controlled for. "We ... teen well-being is due to other aspects of the family ...
... genetic disorders have uncovered insights into those diseases ... divide. Focusing on the cohesin complex, a group ... pairs, scientists have discovered mutations that disrupt cohesin, ... cohesinopathies. "We are learning more about how ...
Cached Medicine News:Health News:Feeling Lackadaisical? Sleep Apnea May Be to Blame 2Health News:Feeling Lackadaisical? Sleep Apnea May Be to Blame 3Health News:Feeling Lackadaisical? Sleep Apnea May Be to Blame 4Health News:Taking Anti-HIV Meds Prior to Exposure May Help Prevent Infection 2Health News:Researchers restore neuron function to brains damaged by Huntington's disease 2Health News:New study shows why swine flu virus develops drug resistance 2Health News:Does dinner make a strong family, or does a strong family make dinner? 2Health News:Mutations impair childhood growth and development by disrupting organization of chromosome pairs 2
(Date:8/21/2014)... R.I. , Aug. 21, 2014 CVS Caremark ... tender results, applicable Reference Yields and consideration payable in connection ... commenced on August 7, 2014 for (1) any and all ... All Notes") and (2) up to a maximum amount of ... 2041 and 5.750% Senior Notes due 2017 (collectively, the "Maximum ...
(Date:8/21/2014)... Aug. 21, 2014  Cyberonics, Inc. (NASDAQ: CYBX ... 2014.  Quarterly highlights 1   ... to the first quarter of fiscal 2014, and other achievements, ... an increase of 6.8%; , Strong international net sales ... currency basis; , U.S. net product sales reached a ...
(Date:8/20/2014)... Aug. 20, 2014  The National Association of ... results achieved by the real-time, pseudoephedrine (PSE) blocking ... Precursor Log Exchange (NPLEx), automatically blocks unlawful PSE ... meth offenders and make arrests. Data released by ... blocked the sale of more than ...
Breaking Medicine Technology:CVS Caremark Corporation Announces Early Results of Tender Offers, Pricing, Election of Early Settlement and Increase in the Maximum Tender Offer Amount 2CVS Caremark Corporation Announces Early Results of Tender Offers, Pricing, Election of Early Settlement and Increase in the Maximum Tender Offer Amount 3CVS Caremark Corporation Announces Early Results of Tender Offers, Pricing, Election of Early Settlement and Increase in the Maximum Tender Offer Amount 4CVS Caremark Corporation Announces Early Results of Tender Offers, Pricing, Election of Early Settlement and Increase in the Maximum Tender Offer Amount 5CVS Caremark Corporation Announces Early Results of Tender Offers, Pricing, Election of Early Settlement and Increase in the Maximum Tender Offer Amount 6Cyberonics Reports Fiscal 2015 First Quarter Results 2Cyberonics Reports Fiscal 2015 First Quarter Results 3Cyberonics Reports Fiscal 2015 First Quarter Results 4Cyberonics Reports Fiscal 2015 First Quarter Results 5Cyberonics Reports Fiscal 2015 First Quarter Results 6Cyberonics Reports Fiscal 2015 First Quarter Results 7Cyberonics Reports Fiscal 2015 First Quarter Results 8Cyberonics Reports Fiscal 2015 First Quarter Results 9Cyberonics Reports Fiscal 2015 First Quarter Results 10Cyberonics Reports Fiscal 2015 First Quarter Results 11Cyberonics Reports Fiscal 2015 First Quarter Results 12Cyberonics Reports Fiscal 2015 First Quarter Results 13Cyberonics Reports Fiscal 2015 First Quarter Results 14Cyberonics Reports Fiscal 2015 First Quarter Results 15Cyberonics Reports Fiscal 2015 First Quarter Results 16Indiana's Stop-Sale System Achieves Significant Results In Battle Against Meth 2
... CITY, Mo., March 16, 2012  Argus Health Systems Inc., ... a National Council for Prescription Drug Programs (NCPDP) focus ... growing biopharmaceuticals industry. John Hill, vice ... NCPDP focus group. The NCPDP focus group, ...
... March 16, 2012 Echo Therapeutics, Inc. (Nasdaq: ... tCGM System as a non-invasive, wireless, transdermal continuous glucose ... drug delivery, today announced financial results for the year ... 10-K is available through Echo,s website at www.echotx.com ...
Cached Medicine Technology:Echo Therapeutics, Inc. Announces 2011 Financial Results 2Echo Therapeutics, Inc. Announces 2011 Financial Results 3Echo Therapeutics, Inc. Announces 2011 Financial Results 4Echo Therapeutics, Inc. Announces 2011 Financial Results 5
Desmarres Retractor Size 4...
Desmarres Lid Retractor thin solid blades. Size 2....
... system introduces a new level ... microsurgical instrumentation. It has an ... with advanced microcomputer control of ... operate with a full range ...
Multiple Modulation Phaco (MMP) programmability with eight surgeon settings for maximum versatility. The combination of the peristaltic power balanced pump and the piezoelectric handpiece results in ...
Medicine Products: