[Jacob-list] Congenital defects - Lysosomal disease

Meg Steensland beegal7 at yahoo.com
Thu Feb 21 14:53:28 EST 2008


Thanks for chiming in Fred. I am a 2003 newbie started w/Jacobs by Eugene Funston in OH. He never had more than a few. I still see his widow or speak on the phone at least weekly as Donna still has two Jacobs-see pix from last newsletter.

I lost 3 sturdy lambs this year - one crashed into the side of a barn while pronging at 2 months and the two rams died in Aug and Nov after losing front end leg mobility. My goat friemd said listerosis-but I suspect something else as antibiotics did nothing and once it started, it just got worse. My first thought was meanie Jeanette hit him and he's lame. Happened last year after one came back from the fair and she knocked him around - he recovered. One of these 2007 rams was born the horrible night of Feb 11 - survived that freeze but died in Aug. The Nov death guy was really sturdy and big and I almost had a buyer for him - glad sale didn't happen or I would have had a worse problem with a new owner.

Here is a link to 2001 discussion of genetic defect studies.

http://www.pairlist.net/pipermail/jacob-list/2001-March/001456.html

Jacobflock at aol.com wrote:
First, I should indeminy myself ... identify myself. I am an aged man with Jacobs.

I received a telephone call from Dr. John Edwards at Texxas A&M's vet school regarding any further status reports on congenital dfects in Jacobs, spefcifically any reports of lysomal disease, GM2 gangliosidosis.

I wrote some iinformation on this lethal disaese in 2002, along with the annual listing of congenital defects in winter 2002, which was made available to the JSC and JSBA. In fact, one of my earliest obervations of this problem was the JSBA meeting iin New York a years ago and there were two reported cases, one confirmed/one unconfirmed in 2003, the last year I managed to remain "on-line".

Columbia University has been working with Texas A&M to try to capture furtherl gene information on this disease (which is also a human disease) anmd both institutions are earnestly lookiing for infected lambs. With new lambs hitting the ground it would be very helpful if Jacob breeders would identify possible infected lambs, monitor thier progress , and when it dies, contact Dr Edwards at 979-845-4608 and see if it is possible to have a necropsy and spinal tissue sample taken.

Most of the affected aheep have Turner 183K as a confirmed pedigree source, one had a Jacobs Ladder as a potential source (Dr. Womack, Txas A&M). Thus, the ability to have a pedigree for the affected lamb would be extremely helpful so the genetic material can be compared.

If you need further informatrion, you might contact Joan Schnieber to see if she has a copy of the article from 2002 which describes the symptoms, etc. It is lethal and many cases of OCD may in fact be GM1 or GM2. If you observe Jacobs with splayed legs, weak pasterns, off gait, ataxia, etc., watch and observe until it becomes recumbant and, if it dies, please get involved ... for the sake of humans and the breed.

Thank you for you attention.

Fred Horak
St. Jude's Farm
1165 E. Lucas Rd.
Lucas, TX 75002




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