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Breakthrough in detection of copper toxicosis


Copper Toxicosis, the hereditary liver disease which has been described as the ‘scourge’ of the Bedlington Terrier, may be eradicated from the breed thanks to a startling new scientific breakthrough announced by the University of Utrecht in the Netherlands.

The official announcement of scientists’ isolation of the defective part of the genome responsible for the disease was made at a special meeting held at the Kennel Club last Friday, involving representatives of the three Bedlington Terrier breed clubs, working Bedlingtons, and the Liver Malfunction Committee, who have spent the past twenty years instigating research and testing into the disease, and officials from the KC’s own Genetic Testing Group.

The meeting heard from Jeff Sampson, the KC’s Genetics Co-ordinator, who stated that, using the Utrecht data, it would take ‘just weeks’ to devise an effective DNA test for Bedlington Terriers, using blood samples, to screen individual dogs for the inherited disease.

The new, definitive test would be able to indicate which dogs were ‘Carriers’, ‘Positives’ or ‘Clears’, and the data from each test could be included on registration paperwork.


Photo by John D Jackson
Bedlington Terriers being judged by OUR DOGS John Holden at Darlington championship show last year.
They are one of the breeds which has benefitted from the research.

Establish

At this stage, the tests will be voluntary, but breeders will be urged, via the breed clubs, the KC and the Liver Malfunction Committee to have their dogs tested, thus to establish breeding bloodlines free of Copper Toxicosis.

The meeting and, indeed, the isolation of the rogue gene marks the culmination of twenty years’ hard work and perseverance on the part of the Liver Malfunction Committee who, for many years, were ‘the voice in the wilderness’ when trying to combat the disease which was literally the scourge of the Bedlington Terrier.

The committee was formed by a group of concerned individuals – all Bedlington breeders and exhibitors – in 1982 who had become aware of the growing problem with Copper Toxicosis in the breed. The prime movers behind the formation of the committee were Fiona Craig and Celia Taylor, together with Stuart & Angela Yearley and others.

The committee wanted to combat the disease head-on and eradicate it from the breed’s various bloodlines.

Initially, the research into the disease was conducted at Cambridge University’s Veterinary School, under the direction of Mike Herrtage, who now is a representative of the KC/BSAVA’s own Genetics Testing group.

In those days the tests involved liver biopsies and, in some sad cases, autopsies on affected dogs. This research enabled Herrtage and his team to devise a reasonably reliable –although very expensive – test to isolate the affected bloodlines.

Stuart Yearley, chairman of the LMC, says: ‘It took time to convince the majority of breeders of the need to conduct these tests. It’s understandable that people don’t like to admit that they might be breeding dogs that are affected in this way. Many in the breed ignored us for a long time and took a lot of persuasion to accept what we were doing.

Eventually, we attached ourselves to the Bedlington Terrier Association and this, if you like, was a way of validating what we were doing.

‘The initial survey conducted at Cambridge indicated that over 30% of dogs were affected and the majority of the remainder were carriers. But through the testing programme, which we eventually managed to convince breeders to take up, a small number of ‘clear’ animals were identified with an accuracy rate of 98.5%.’

The Kennel Club refused to record any testing details in pedigrees and registrations until a 100% effective test could be devised. However, the University of Michigan, partly funded by the LMC, created a Genetic Marker test which made screening more accessible. Through the new screening programme over 600 Bedlingtons in the UK alone were tested.

It was then that the designation of ‘1’ for a ‘clear’ gene was created and ‘2’ for an affected gene. Thus the best definition for a dog would be 1.1 for a ‘Clear dog’, whereas 1.2 would be a ‘Carrier’ and a 2.2 dog would normally be a fully affected dog.

Last year, using the Michigan test as a basis for their research, the University of Utrecht, Holland, devised their own test and managed to isolate the defective part of the genome responsible for Copper Toxicosis – and the results of this were announced to the KC meeting just weeks later in January 2002.

Organise‘The meeting of the KC and breed clubs was arranged some time ago to try to organise a timescale for blood testing for Copper Toxicosis,’ says Stuart Yearley, ‘However, we were hopeful that we might have an announcement to make from Utrecht, and indeed we did. We hope that the test, once devised, will be licensed for use in the UK by the Animal Health Trust.’

It was agreed at the meeting that it would be necessary to have ‘a big voluntary take-up of the test by breeders over the next two years’ before it was feasible to consider what further action should be taken, and that this proposal would be conveyed to representatives’ respective breed clubs for discussion. It was envisaged that all such test results would be monitored and could be made available for inclusion in registration documentation. Stuart continues: ‘We also agreed that DNA Profiling for identification purposes should be included on registration documents. This represents a major step forward and is very positive that everybody is in agreement over this.’

Breeders and exhibitors should be grateful to the efforts of the Liver Malfunction Committee over the past twenty years. In that time, the Committee raised over £10,000 to subsidise tests for those breeders who could not afford to pay for their dogs to be tested, and to help fund vital research – such as that at Utrecht – which helped to speed up the process in isolating the affected part of the genome.

Funds were raised by raffles, tombolas, exemption shows organised by the committee and some very generous individual donations.

‘It’s mainly through the work of the ‘little old ladies’ that we’ve got to where we are today,’ says Stuart Yearley. ‘It’s good for the breed and hopefully, one day, we will see 100% clear bloodlines and the scourge of Copper Toxicosis will be eliminated forever.

‘It’s a good start to the year!’