NCL in the Australian Cattle Dog


I am a regular owner of an Australian Cattle Dog, I do not have any
education relating to the subject. I will tell about the disease, as I
myself understand it with my common sense. There may be errors in my text.
Intensive research is done about this all the time, so there may be new
information which I have not received.



What is NCL?

NCL is an abbreviation of the word Neural Ceroid Lipofuscinosis. The Ceroid
Lipofuscinosis, CL, is met in many breeds including Border Collie, Cocker
Spaniel, English Setter, Saluki, Chihuahua and Dachshund . A gene test
has been developed to determine sick dogs and carriers of the disease
for at least Border Collies. NCL is met in other species and even in humans.
There are many mutations of the NCL, of which many are known.
CL is a rare variation of epilepsy. It is a metabolic disease which particularly
affects nerve cells. Sick dogs suffer from enzyme shortage, which causes
some of the body’s lysosomal storage materials, ceroid and lipofuscin,
to collect to cells of the tissue instead of dissolving at the enzyme’s
impact and being transported out of the system harmlessly. These substances
are mainly accumulated in the nervous system, particularly neurons, which
causes the cells to eventually explode and expire. The CL is not an infectious
disease. Dogs which suffer from this disease are born ill, although they
do not yet express symptoms. The effects of the enzyme deficiency may
appear only after the dog has grown up.



Is there a cure for NCL?

Unfortunately, there is no cure known for the disease. It always leads
to death, although dogs often need to be put to sleep due to severe behavioural
problems even before this, often less than a year since the starting of
the symptoms.
How common is NCL in cattle dogs?
I do not have confirmed information from other countries, but in Finland
there has been only two confirmed cases to the date of this article. Thanks
to those owners, who asked an autopsy for their dogs. Now we can fight
this disease, because we know of its existence. The Finnish Australian
Cattle Dog Club is working with gene researchers in order to develop a
gene test for this disease. There are also statistics made on the basis
of the two confirmed NCL-cases on possible inheritables, statistics are
shown further below.


The beginning of NCL symptoms and signs

Unfortunately there is no one certain symptom which could be an identifier
of the NCL. The symptoms can have many variations, depending on the breed,
the dog and its original characteristics. Thus, even within the same breed
there can be dogs with very different symptoms. The disease is believed
to be very painful when progressing, which can be observed as varying,
increasingly bad symptoms. The only certain thing is that the disease
will get worse, and after the first symptoms are shown, the disease can
progress rapidly toward the final destination, death.
Most of the dogs can be symptomless up to the age of 15-24 months, and
in some breeds even up to 4-6 years. However, it has been claimed that
the dogs which are put to sleep at 5-6 years for severe symptoms have
most likely had some symptoms even before the age of 3 years, but they
have not been identified until they’ve got worse.
One of the symptoms can be an increased, periodical restlessness and aimless
wandering. As the disease progresses the symptoms get worse and the time
between periods shorten. Eventually the dog wanders in a manic manner,
being unable to relax. Abnormal fear of touch can be observed, for example
putting on the collar can cause avoidance and aggressive protests as the
dog fears the pain caused by the procedure. Also combing the dog can become
impossible. The dog can have problems moving normally. Sitting normally,
jumping or climbing can cause unreasonable problems. The dog might also
have compulsive movements. Also symptoms of dementia can be present, and
the dog might forget the house training he has already learned. The dog
might show blind rage or stare at a certain spot increasingly long periods
of time, even hours as the disease progresses. Unreasonable fear reactions
can occur, and the dog can become afraid of things which have been safe
and familiar to it. The dog can overreact to the owner’s commands
and gestures and even glances. The dog might have eyesight failures. The
dog can become more and more aggressive, eventually biting with blind
rage without being unable to control its own behaviour.


Inheritance of the NCL

The NCL is believed to be inherited recessively. It means that both parents
need to be at least carriers of the disease, so that some of the puppies
will become sick. The fact that a dog is a carrier means that the dog
itself has no symptoms, but it carries one of the mutation gene copies
which cause the disease. If one of the parents is completely healthy,
without having the mutation gene, none of the puppies will become sick.
There can be symptomless, healthy carriers of the disease in the litter,
if one of the parents is either sick or a carrier of the disease.

Some examples of the inheritance of the CL:

Dog A

is healthy. It doesn’t posses the gene which causes the disease
in its inheritance, so the offsprings can’t become sick, even when
the other parent is sick or a carrier, ie. possess the sick gene. When
combined with dogs B or C, either half or all of the offsprings are healthy
carriers of the disease.

Dog
B
is a healthy carrier of the gene. It will not get sick,
but it still carriers one copy of the gene wich causes the disease in
its inheritance. If this dog is combined with dog A, the offsprings can
not be sick, but about a half of them will be carriers. If the dog is
combined with either B or even C, even half of the offsprings can be sick.

Dog
C carries two copies of the gene which causes the disease
and is thus born sick, although it will only start presenting symptoms
approximately at ½ to 1½ years. If this dog would be used
with dog A, all of the offsprings will be healthy carriers of the disease.
If this dog is combined with dog B, half of he offsprings would be healthy
carriers and the other half sick dogs presenting symptoms.

What happens
when two healthy carriers are combined: Statistically half of the offsprings
are healthy carriers, ¼ are healthy and ¼ are sick, as presented
in the picture below:

When a healthy carrier is combined with a sick dog, half of the offsprings
are healthy carriers and half are born sick, as presented in the picture
below:

When a carrier dog is combined with a healthy dog, none of the puppies
are sick and half are carriers, as presented in the picture below. This
way the carriers do not need to be counted out of breeding, when they
are combined with a genetically healthy individual.

A list of the dogs who possibly possess the NCL-gene, based on the 2 dogs
which have been diagnosed sick:

Note!
Healthy carriers of the disease can be used in breeding as soon as the
gene test is developed. Healthy partners can be chosen for the carriers!!

Confirmed carriers (sick offsprings):
· Cattlefarm’s Darra Power

· Cattlefarm’s Magnum Max
· Cossaks Runaway Ringa
· Cattlefarm’s Wild Wind
Presumed carriers (siblings of the sick dog 50 % probability to be carriers):

· Cattlefarm’s 16.8.2003 born litter
(Blu Hat Trick, Red Flabbergast, Red Pieceofcake, Fullofwhims, Blu Hole
in one, Red Hot Gossip)
· Cossaks 2.5.2004 born litter
(Top Secret, Touch of Gold, True Unity, Twilight Moon, Twinkling Eye)

Siblings of the confirmed carriers: (62,5 % probability to be carrier):

· Cattlefarm’s 8.6.1997 born litter
(Hoped Blue Boy, Clever Candy, Shameless Lass)
· Cattlefarm’s 9.7.1999 born litter
(Gun Keeper, Safe Shadow, Will Power)
· Cossaks 25.11.2001 born litter

(Roadrunner Boy, Rambling Rose, Ready Steady Go, Rock’n’Roll,
Rolling Fire)
Possible carriers (straight line to a mutual grandparent):
· Cattlefarm’s Blue Pirate
· Cattlefarm’s Blue Dynamo

· Cattlefarm’s Wild Wilma
· Reddenblu’s Gunpowder
Mutual grandparents:
· Cattlefarm’s Blue Rock Boots

· Reddenblu’s The Proclaimer
· Turrella Blue Dynamite
MPBC* calculated relatives, making lines to them (= both parents possess
the same
grandparent) should preferably be avoided until we get the gene test for
NCL:
NCL-confirmed dog(1):

Cattlefarm’s Blue Rock Boots 25,00 %
Turrella Blue Dynamite 25,00 %
Turrella Blue Pirate 18,75 %
Dalotek Blue Dynamite 18,75 %
Taits Glen Red Sonny 15,82 %
Reddenblu’s Navaho Rug 15,63 %

Turrella Blue Stetson 12,50 %
Reddenblu’s North of the Law 11,72 %
Dreamtime Fallen Angel 11,72 %
Turrella Blue Pebbles 9,38 %
Turrella Blue Arrow 9,38 %
Jandanee Blue Mist 9,38 %

NCL-confirmed dog(2):
Cattlefarm’s Blue Rock Boots 37,50 %
Reddenblu’s The Proclaimer 25,00 %
Reddenblu’s Navaho Rug 21,88 %
Turrella Blue Stetson 18,75 %

Reddenblu’s North of the Law 17,97 %
Dreamtime Fallen Angel 17,97 %
Turrella Blue Pirate 15,63 %
Dalotek Blue Dynamite 15,63 %
Reddenblu’s Angel Luvs Badmen 14,06 %

Taits Glen Red Sonny 13,18 %
HGF A-bit-O Blu Jk’s Cowchip 12,78 %
Hanging Grove Farm Kit 9,38 %

*Major percentage
of blood contributors aka percentage of blood. Calculated with DalPedigree
program from 10 generations (Willis, 1989).

How do I find out if my dog has NCL?

At the moment it is hard to determine if a living dog has NCL. When the
dog already presents symptoms, the disease can be seen in the official
eye examination. At this point, the disease can also possibly be noticed
in the magnetic resonance imaging. The certain diagnosis can be given
only with an autopsy. If the dog’s behaviour is abnormal, the owner
should contact a vet who is specialised in neurology. If the dog is presenting
symptoms of the NCL, the symptoms will certainly get worse over time.
If a dog is put to sleep because of suspected NCL symptoms, an autopsy
should be done. The autopsy doesn’t cost much, but can bring much
more information about the causes of the dog’s behaviour.


NCL in the future of the ACDs

When the gene test for the NCL in ACDs is developed, the disease will
hopefully eventually disappears. The breeders can select the dogs used
in breeding so that sick dogs are no longer born. The carriers can be
used in breeding to ensure that the gene pool remains broad enough. ACD
people all around Finland have been active in gathering blood samples
for researchers in order to develop the gene test. Still more blood samples
are needed and all dogs who are put to sleep because of suspected NCL
symptoms should be done an autopsy. Until the gene test is developed,
examining the pedigrees is the best way to breed healthy dogs. In the
future there is hope even regarding this fairly rare disease.

Original text by: Anna Elvilä-Moksi

Translated by: Elli Kinnunen, Anna Elvilä-Moksi
Sources: www.caninegeneticdiseases.ne

 
copyright
© Doris Duewel, all rights
reserved / webmaster : Doris

 

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