[Jacob-list] GB item: G444R mutation (Tay-Sachs) in Jacob sheep

[gotothewhip at aol.com]

I was the one who went thru a Tay-Sachs false alarm.
 
We had a lamb born out of a purchased ewe bred to our old herdsire, Lillywold Job
 
The ewe had a few lambs for us previously, with no trouble
 
She had twins early one morning, we missed the delivery, but they were still wet when we found them.  I made sure both nursed, but noticed one was a bit wobbly and seemed to have trouble standing up.  The next morning it was apparent he had not yet stood.. We brought him in the house.. tubed him with Clostrum and got him going.
 
He did not stand on all 4 feet for 2 weeks.  We were ready to put him down when he stood up and began walking.. He had a wobble to his gait  see the folowing videos: http://www.youtube.com/watch?v=aWaXNV8s5Ks  and http://www.youtube.com/watch?v=2624D625Vdk 

We had put a lot into this lamb... Not just to try to get him to get over whatever his issue was... I was determined to find an answer for what was going on.. so I would know if it was a genetic, nutritional or other issue.. so if it was something I could prevent... I would...He had every *just in case* treatment that any of the small ruminant specialists at CSU could come up with.  Vitamin Injections mineral supplements, antibiotics, steroids.. you name it...... We took it too far in his treatment, but I NEEDED an answer as I was hiping to know how to prevent this from happening again to me or anyone else.

Anyhow.. little Wobbles underwent a battery of tests.  MRI, Ultrasounds, Radiographs.. LOTS of Bloodwork.. He was slowly making progress.. walking well and pretty straight, and then one weekend, it went downhill FAST... he had what appeared to be a seizure.

We pulled another round of blood and put him down.. I took him to CSU immediatley for a necropsy.  They pulled the tissues requested, and with the blood samples I had.. They overnighted the tissues and blood to NYU, where they were evaluated.  We also had the skull looked at for OCD and it was normal and fine.

We do not know anymore today than we did the day he was born.  Liver showed a SLIGHT deficency in copper.. but his symptoms do not match a CU deficiency.  His twin brother was blood tested (not as accurate as liver biopsy) but he tested in the normal range.  He turned out to be a stunning ram and was Grand CH ram at Estes Park Wool Market in 2010.

I think many folks thought I was a little nutty when I publically asked about OCD in lambs, as it wasnt something to be talked about, and seemed to be something nobody admitted to knowing anything about.  While Wobbles didnt have OCD.... I think the situation was something that needed to be talked about.  Im an Extension Agent by trade, and just dont like sweeping things under the rug.. I think talking about things is the best way to figure out the puzzle.

Jennifer Tucker
www.MooseMtnRanch.com




-----Original Message-----
From: Linda <patchworkfibers at windstream.net>
To: Jacobflock at aol.com
Cc: Jacob-list at jacobsheep.com; gotothewhip at aol.com; FHorak at aol.com
Sent: Fri, Feb 18, 2011 4:16 pm
Subject: Re: [Jacob-list] GB item: G444R mutation (Tay-Sachs) in Jacob sheep


I am not alarmed, but wonder if you might share with us early signs of TSD in lambs as well as when we might expect to see signs. I believe a concerned breeder in CO had a lamb tested for TSD. The lamb was negative and the results showed a copper deficiency or some such, I think. J - jump in here, please. We do need to be aware of TSD, whether or not we tested, and we also have to be aware of other possibilities for the symptoms.

Turner 183K shows up in the background of every major flock in the country if you merge JSC and JSBA records.

Linda



Jacobflock at aol.com wrote: 
Great Britain Listers, Jean Blacknell and JSS members - The Jacob (American) testing for the Tay-Sachs mutation has hit the 100 carrier mark out of 561 tests; the common source continues to be Turner 183K; son of imports Trevor UK 326 and Limper B97 GB2111.  The mutation in North America has been reviewed by the JSBA and they have considered this lethal congenital neurological problem to be non-concerning.  Following JSBA counsel, Jacob breeders in North America apparently need not be alarmed.  
 
Jacob breeders in GB might be interested in a developing study; Jean Blacknell, Field Supervisor, has been aware of possible GM2 in Jacobs for about two years.  For GB breeders .... I received the following note from England today: .....  I was interested in the Vet Path paper on the GM2 problem.  We identified an LSD Jacob sheep in the UK and in cooperation with Guys Hospital ran plasma for HexA and Hex B and discovered  the TSD variant in the Jacob.  The flock is quite small (100 ewes, rams and lambs) but had two confirmed cases of LSD.  I note the high carraige of the mutation in the US but no records of importation from the US.  Coincidence? ......  Mark.  
 
I told Mark that we think the source is the UK and our US incidence is through Turner 183K.  
 
Regards
Fred Horak
St. Jude's Farm
1165 E. Lucas Road
Lucas, TX  75002
Ph 972-727-0900
 
 

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Patchwork Farm Jacob Sheep 

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