[Fwd: Research Update #10 from ? National Office]

[Guest guest]

This exciting news from the ALS org., It is great for PLS and others

aswell

Reyerse

Orginazation

--- Research Update #10 from ? National Office

Date: Tue, 08 Feb 2005 09:17:39 -0800

Reply-To: The ALS Association

To: jreyerse@...

ALSA Funds New Investigator-Initiated Projects for 2005

The ALS Association (ALSA) has funded the following projects toward

finding a successful treatment for ALS and is pleased to announce that

among the current projects is one that is a partnership with The

Amyotrophic Lateral Sclerosis Society of Canada (ALS Canada). Another

partnership is with ALS Therapy Alliance in Massachusetts for two

additional grantees. Five starter grants, funded at a total of $252,000,

and five multiyear grants totaling $817,169 result in a total commitment

for ALSA of $1,069,169. The investigators funded propose exciting lines of

inquiry that should open up avenues to new therapeutics.

Strategies include finding out more about the components of muscle and

nerve that may contribute to the disease, structure of the mutant SOD1

protein, dynamics within the neuron that are implicated in the disease,

and inflammatory aspects that might influence ALS. Significantly, ALSA

has funded a new effort toward a national registry of ALS patients.

Muscle and Nerve Contributors

The muscle itself may be a prime source of disease in ALS, and a new

avenue of research proposes to investigate this idea further. Chien-Ping

Ko (see http://www.alsa.org/research/grant.cfm?id=594) and coworkers find

that changes appear in muscle long before any other sign of ALS in the

SOD1 mouse model. This group will carry out further manipulations to

decide what role muscle tissue plays in the disease, and indeed, if muscle

changes instigate the entire process.

A new suspect in ALS prompts Jean-Pierre n (see

http://www.alsa.org/research/grant.cfm?id=593) and colleagues to delve

further into the role played by chromogranins. These components of

certain nerve cells appear to associate with the mutated ALS enzyme, SOD1.

New ALS mutant mice will be made that make more than normal amounts of

chromogranins, to see if the disease symptoms worsen. The cellular

protectors called chaperones may also have roles in ALS. Funding of this

project is in partnership with ALS Canada.

The cellular protectors called chaperones may also have roles in ALS.

Zuoshang Xu (see http://www.alsa.org/research/grant.cfm?id=599) and

colleagues will see if the scarcity of chaperones in motor neurons is what

makes these cells so susceptible to damage in ALS. The scientists seek

the exact chaperone that interacts with mutant SOD1, so that future

experiments can specifically manipulate it to alter the disease. This

project is co-funded by ALS Therapy Alliance in Massachusetts.

Mutant SOD1 Structure

At the most fundamental level, we know the mutated SOD1 molecule can

produce ALS, probably by interactions with other proteins in the cell.

Ashutosh Tiwari (see http://www.alsa.org/research/grant.cfm?id=598) and

coworkers will try to find out the precise physical properties of the

mutant protein that cause toxicity. Detailed studies on the molecule are

planned, including mass spectroscopy. This project is co-funded by ALS

Therapy Alliance in Massachusetts.

Nerve Cell Dynamics

Mitochondria are the power plants of all living cells. These cell

organelles also orchestrate cell death. Jian Liu (see

http://www.alsa.org/research/grant.cfm?id=595) and colleagues plan to find

out more about what goes wrong with mitochondria in the spinal cord of ALS

mice. Gunther (see http://www.alsa.org/research/grant.cfm?id=592)

and colleagues will look for changes in mitochondrial function in a new

microbial model system. They have introduced the SOD1 mutation into a

strain of yeast that can be studied efficiently to dissect the exact

mechanism of how mitochondrial function might be disrupted by the ALS

mutation. They will then expand their studies to a mammalian cell model.

Grierson (see http://www.alsa.org/research/grant.cfm?id=591) and

colleagues will seek defects in the inner cellular transportation system

that allows motor neurons to carry out their demanding tasks. These are

the nerve cells affected by ALS. The researchers intend to tag and

directly observe the molecular means by which motor neurons move their

cellular cargoes, and how the SOD1 mutation might hinder the job.

Inflammatory Aspects

Moeller (see http://www.alsa.org/research/grant.cfm?id=596) and

coworkers find that SOD1 mutant mice have changes in the way their brain's

immune cells work. These microglia are increasingly suspect in the

disease. The investigators will directly test how microglia and motor

neurons isolated from ALS mice interact to change each others' function

and survival. Inflammatory processes within the nervous system are also

the focus of Beattie (see

http://www.alsa.org/research/grant.cfm?id=590) and colleagues, who find,

in the brain, that excitatory transmission is changed through an

inflammatory mediator, tumor necrosis factor (TNF) alpha. Apparently, TNF

alpha acts to increase the likelihood that too much excitation can damage

or kill neurons. The researchers will see if the relationship holds for

motor neurons of the spinal cord.

National Registry

Finally, funds from ALSA will further the work of a consortium of

epidemiologic investigators that will serve scientists working on the

genetic and environmental influences that may produce the disease. Lorene

(see http://www.alsa.org/research/grant.cfm?id=597) and colleagues

intend to develop standardized means to collect data on patients and their

families, to pool the data and put the information at the easy reach of

all investigators. A shared web site would serve as the pivotal point for

all genetic and epidemiologic studies of the disease. ALSA is pleased to

support what may contribute to a national registry of ALS patients and

families.

*****************

The ALS Association, National Office

27001 Agoura Road, Suite 150

Calabasas Hills, CA 91301-5104

Phone:

Fax:

Each year since its founding in 1985, The ALS Association has increased

its investment in research grants. Of the more than $25 million raised

during the last decade in the quest to unlock the mysteries of ALS, each

dollar has gone directly to investigators doing the research. Read more at

www.alsa.org/research.

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