Grant: Doctoral Research Award - Priority Announcement: Huntington Disease
McMaster University
$0.00
The Huntington's Disease protein has a role in the nuclear stress response: Mechanism for a Huntington's Disease Biomarker
FY 2008-09
Summary
Huntington's Disease (HD) is a genetic neurological disorder that affects families worldwide. HD is caused by a single gene encoding the huntingtin protein (htt) and an affected individual has a 50% chance of passing this gene onto any offspring. HD manifests with physical, cognitive and emotional symptoms caused by degeneration of neurons in the brain leading to death within 10-20 years post-onset. There is currently no treatment or cure for HD. The causal factor of HD, mutant htt, has been studied for over a decade, however there has been no success in finding drugs to cure HD. This is largely due to difficulties in studying the normal function of htt, making it impossible to ascertain what is going wrong in an affected individual. Our labs focus is on studying the normal function of htt, in hopes of finding novel drug targets for this disease. There are other similar neurological disorders, including Alzheimer's Disease (AD) and Parkinson's Disease. These disease have different causal factors, however, all three diseases are related since their main pathology is degeneration of neurons in the brain; indicating that there may be similar things going wrong. To this end, I have recently shown that htt interacts with the protein cofilin which is involved in rescuing a cell from stress. The mis-regulation of cofilin has been shown to be involved in the neuronal degeneration seen in AD. I have been able to show that this protein is also mis-regulated in HD and may be involved in neuronal degeneration in a similar manner to AD. I have further observed that changes in the cofilin protein can be tracked in HD patient blood samples through disease onset making cofilin protein levels a biomarker for HD. Current efforts are underway to deduce if changes in cofilin protein levels in the blood will be useful as a biomarker for AD. Currently there are no biomarkers for neurological diseases, making this research a milestone in drug discovery for both HD and AD.
Department
Program
Doctoral Research Award - Priority Announcement: Huntington Disease
Recipient
Location
Ontario, Canada
Recipient Type
Academia
Type
Grant
Source
CIHR Funding Decisions Database
As published
Id
178030
Name
Munsie Lise N
Region
Ontario
Country
Canada
Orgtype
Institution Paid; Research Institution
Partnername
Huntington Society of Canada
Instname2
Genetics
Theme2
Biomedical
Prcname2
Doctoral Research Awards - A
Projecttitle
The Huntington's Disease protein has a role in the nuclear stress response: Mechanism for a Huntington's Disease Biomarker
Programtype2
Studentship Programs
Orgnameinp2
McMaster University
Cihramount2
$0
Cihrequipment2
$0
Programname2
Doctoral Research Award - Priority Announcement: Huntington Disease
Keyworddelim
Alzheimer'S Disease; Biochemistry; Biomarker; Biophotonics; Cell Biology; Chemical Genetics; Drug Discovery; Huntington'S Disease; Live Cell Imaging; Tissue Culture Model
Abstract
Huntington's Disease (HD) is a genetic neurological disorder that affects families worldwide. HD is caused by a single gene encoding the huntingtin protein (htt) and an affected individual has a 50% chance of passing this gene onto any offspring. HD manifests with physical, cognitive and emotional symptoms caused by degeneration of neurons in the brain leading to death within 10-20 years post-onset. There is currently no treatment or cure for HD. The causal factor of HD, mutant htt, has been studied for over a decade, however there has been no success in finding drugs to cure HD. This is largely due to difficulties in studying the normal function of htt, making it impossible to ascertain what is going wrong in an affected individual. Our labs focus is on studying the normal function of htt, in hopes of finding novel drug targets for this disease. There are other similar neurological disorders, including Alzheimer's Disease (AD) and Parkinson's Disease. These disease have different causal factors, however, all three diseases are related since their main pathology is degeneration of neurons in the brain; indicating that there may be similar things going wrong. To this end, I have recently shown that htt interacts with the protein cofilin which is involved in rescuing a cell from stress. The mis-regulation of cofilin has been shown to be involved in the neuronal degeneration seen in AD. I have been able to show that this protein is also mis-regulated in HD and may be involved in neuronal degeneration in a similar manner to AD. I have further observed that changes in the cofilin protein can be tracked in HD patient blood samples through disease onset making cofilin protein levels a biomarker for HD. Current efforts are underway to deduce if changes in cofilin protein levels in the blood will be useful as a biomarker for AD. Currently there are no biomarkers for neurological diseases, making this research a milestone in drug discovery for both HD and AD.
Competitiondate
200810
Approvedterm2
1 yr 0 mth
Pinamesdelim
Munsie, Lise N
Approvedterm
1 an 0 mois
