Award details

Neuroprotection by the WildS gene in models of ALS

Reference	BBS/E/B/0000F103
Principal Investigator / Supervisor	Professor Michael Philip Coleman
Co-Investigators / Co-Supervisors
Institution	Babraham Institute
Department	Babraham Institute Department
Funding type	Research
Value (£)	2,279
Status	Completed
Type	Institute Project
Start date	01/08/2002
End date	31/07/2005
Duration	36 months

Abstract

Amyotrophic Lateral Sclerosis (ALS) is characterized by progressive dysfunction of the motor system that results in muscle weakness and ultimately death. Weakness is due to disconnection of skeletal muscles from their corresponding motor neurons, an event that is generally regarded to be the result of motor neuron death. Several published report, however, suggest that the symptoms and signs of ALS may precede the loss of motor neurons. These findings raise the question of whether the nerve fiber (axon) itself may degenerate before the death of motor neurons, causing weakness by eliminating the essential connection between motor neuron and muscle. Thus, prevention of axon degeneration in ALS may improve strength and delay death. This proposal uses the unique WldS mouse mutant to test this hypothesis. The WldS is resistant to nerve fiber degeneration, and the WldS gene may be protective against motor neuron death. We will introduce this gene into two mouse models of ALS to test whether the WldS gene will protect against 1) motor neuron death; 2) nerve fiber death; and 3) death of the animal. These studies will provide important new information regarding the role of nerve fiber degeneration in ALS, and also demonstrate if the WldS gene can protect against motor neuron disease.

Summary

unavailable

Committee	Closed Committee - Animal Sciences (AS)
Research Topics	X – not assigned to a current Research Topic
Research Priority	X – Research Priority information not available
Research Initiative	X - not in an Initiative
Funding Scheme	X – not Funded via a specific Funding Scheme

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