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PRA

Donation

Gathering

2007 PRA UPDATE

Previously:

PRA is an inherited eye disease , affected dogs to go blind. There are many different forms of PRA; for some of these the genetic cause has been identified and tests are available to help breeders to prevent affected pups being produced and eliminate the disease from their breed. For many other forms of PRA, however, the genetic cause is unknown, and there is no known cure.

Although it had most certainly existed in our breed long ago, PRA was
first clinically diagnosed in Glen of Imaal Terriers in the summer of
1996. In 1997, Maura High of the Glen of Imaal Terrier Club of
America convinced world-renowned canine eye researcher and geneticist
Dr. Gregory Acland of Cornell University to study PRA in Glens. Dr.
Acland and his team have been working on this problem for us ever
since. As special and unique (and stubborn) as our breed is, so is
their form of PRA. In Glens, PRA is late onset, with disease first
detectable by ophthalmologic examination usually between the ages of 3
and 7. Over the years many owners around the world have diligently
had their Glens eyes tested, shared the results with the Glen
international database, and submitted blood samples for research.

Ten years ago genetic sequencing involved many slow and tedious
man-hours, but technology is now rapidly improving. What used to take
Dr. Acland months to accomplish can now be done in a matter of hours.
Hence, there is hope. As you will see from the following article, Dr.
Acland is ready to start a very exciting project in our breed's
research, and he needs our help.

And Now:

On September 8, 2007, Dr. Acland came to the Glen Gathering in Barre, Massachusetts, where he tested 31 dogs. We held a 50/50 raffle with the proceeds to go to Dr. Acland's research. The raffle winner, Hilary Stark, graciously donated her portion back to Dr. Acland, so the raffle raised $115 towards eye research. Altogether, including 100% of the eye clinic fees, the Gathering raised $734 for Dr. Acland. That's almost 2 chips! (see below)

          Dr. Acland updated us on the progress of his research. One exciting development is that for most dogs, we can now use cheek swabs to collect a useful DNA sample. This is much simpler and quicker than blood collection, and even an owner can collect and submit a sample. (Blood samples are still preferred for affected dogs.)

          Dr. Acland told us that Glen PRA continues to be a very puzzling disease, unlike any other PRA known in dogs. While most PRA affects the rod cells in the retina first, diminishing night vision, in Glens the cone cells get sick first, which should initially degrade daytime vision. Observations by owners of affected dogs when the symptoms begin to be apparent would be appreciated. Contact Ara Lynn or Maura High for this.

          The mode of inheritance of Glen PRA is still uncertain. It would be useful to examine and take a DNA sample from another terrier or Irish breed with PRA, especially a Soft-Coated Wheaten terrier, Kerry Blue, or Irish terrier. Dr. Acland has heard of PRA in those breeds, but has never seen one. Again, if anyone can help locate such a dog, it would be greatly appreciated and might shed some more light on this disease.

Follow-up on the Canine Genome Project:

Two years ago Glen of Imaal Terriers participated in a portion of the Canine Genome Project at Broad Institute, MIT. We searched the world over with the goal of finding 20 Glens each with 4 distinct grandparents. We were unable to quite reach that goal, because so many of the Glens had overlapping grandparents. In the end, although we came up with 28 of the most-distantly-related-as-possible individuals from three continents, these represented only 95 (instead of 112) different grandparents. Glen of Imaal Terriers proved to be the most homozygous of all the breeds analyzed, meaning that genetically, Glens are far more similar to each other than is the case for any other breed. Glens' participation in the project was particularly useful for studies of dog relatedness in Dr. Elaine Ostrander's laboratory at the National Institutes of Health. Although it didn't also find our defective PRA gene for us, it did provide a nice pool of control dogs' DNA to which Dr. Acland has access.

DNA Chips:

Technology is beginning to catch up to research efforts. DNA chips are being developed that can read DNA instead of computer code. This is a huge improvement over slower and more labor-intensive methods of analyzing DNA that Dr. Acland has had to use in the past. These chips should eventually be capable of reading a DNA sample and scanning it for a panel of thousands of diseases. Currently, researchers are using the chips to try to identify regions in DNA code associated with various specific diseases. The chips are expensive, costing about $250 each and $150-200 to run the chip through the testing program.

Dr. Acland has established a collaboration with Broad Institute, MIT, in an attempt to make such studies more cost-efficient, to determine if pooling the DNA from affected dogs onto one chip and from unaffected dogs onto another chip, might identify consistent genetic differences between the two groups. This collaboration is funded, in part, by the Morris Animal Foundation, and if it works will make association mapping studies for many breeds, including the Glen of Imaal Terrier, much more cost effective. In theory, it should work, but in practice so far the computer that ran the test program got confused by so many variations between the individual dogs' DNA, and it could not read the chips. So, this might work in the future, but for now, to tackle the Glen of Imaal PRA problem, Dr. Acland believes that we should go ahead with what works currently, and use an individual chip for each dog. There are several advantages to using this approach, not the least that it is now well-proven technology.

The problem is that the minimum order from the chip manufacturer is 90 chips. (There is a price break at 200 chips.) So far Cornell University has been reluctant to make this investment. Ninety chips is far more than Dr. Acland needs for Glens, but he is hoping to get a couple of other breeds to pitch in and help buy chips for their own breeds' research. For Glens, he would like to start with 20 chips; for 10 affected dogs and 10 controls. Then, he would like to repeat the process using another 10 affected dogs and another 10 controls. We are very excited at the prospect of using these chips; it would make a giant leap forward in Glen research.

Our DNA Chip Fund Raiser

Dr. Acland is applying for partial grant funding towards this project, but we would like to speed things along and help ensure that our Glen problem is tackled first.

So, we've embarked on a chip fundraising drive. The goal is in two stages: at least $8,000 by November 1, 2007, and another $8,000, for a total of $16,000, by December 31. Please make a donation, large or small, to help solve the Glen PRA problem. Your donation is tax deductible when your check is made payable to the

James A. Baker Institute at Cornell University

with a notation on the check or an accompanying letter stating that the money is to be used specifically for Dr. Acland's research into Glen of Imaal Terrier PRA.

Because it is easier for the staff at James A Baker Institute to receive the money all at once (so they can focus on eye research instead of keeping track of lots of checks trickling in), we have asked Kathy Georgianna, who has collected and forwarded money to Dr. Acland in the past, to do so again in this fund-raising drive. Please mail your check by Dec 31 to:

Kathy Georgianna
1070 B West Road
Williamsburg, MA 01906

Printable Coupon

or, if you prefer, you may mail your check to the GITCA treasurer:

Les Anthony
8 Nutmeg Court
Wallingford, CT 06492

If you have any further questions please contact Maura High, chair of the GITCA Health Committee, or Ara Lynn.

Glen Gathering Newsletter & PRA:

Ara Lynn lynn.ara@gmail.com

202 Poor Farm Road
New Ipswich, NH 03071 USA

1-603-878-3552

PRA:

Maura High coleraine@centurytel.net

21366 McCoy Road

Lawson, MO 64062 USA

1-816-296-7030