Groote Eylandt, a large island in the Gulf of Carpentaria, has the highest incidence of Machado-Joseph disease in the world. Some 5% of inhabitants have this inherited disease.

Machado-Joseph disease is a degenerative disease that affects muscle control. In severe cases, sufferers lose the ability to speak or walk. It is a dominantly inherited disease. If one person with MJD reproduces with another person without it, there is a 50% chance their offspring will inherit the condition. There’s no cure or means of delaying the onset, and so treatment revolves around helping sufferers live with the symptoms.

There are two fairly distinct strains, one of which originates in the North Atlantic and is a major disease in the Azores. The other is associated with Asia. It was at one time thought that the presence of MJD on Groote Eylandt was due to contact with Portuguese sailors, but more recent testing indicates that it is from the same haplogroup as the cases found in Japan, Taiwan and India, so it may be due to early contact between Makassan fishermen and the people of Groote Eylandt.

Mutational Origin of Machado-Joseph Disease in the Australian Aboriginal Communities of Groote Eylandt and Yirrkala

dv said:

Groote Eylandt, a large island in the Gulf of Carpentaria, has the highest incidence of Machado-Joseph disease in the world. Some 5% of inhabitants have this inherited disease.

Machado-Joseph disease is a degenerative disease that affects muscle control. In severe cases, sufferers lose the ability to speak or walk. It is a dominantly inherited disease. If one person with MJD reproduces with another person without it, there is a 50% chance their offspring will inherit the condition. There’s no cure or means of delaying the onset, and so treatment revolves around helping sufferers live with the symptoms.

There are two fairly distinct strains, one of which originates in the North Atlantic and is a major disease in the Azores. The other is associated with Asia. It was at one time thought that the presence of MJD on Groote Eylandt was due to contact with Portuguese sailors, but more recent testing indicates that it is from the same haplogroup as the cases found in Japan, Taiwan and India, so it may be due to early contact between Makassan fishermen and the people of Groote Eylandt.

Mutational Origin of Machado-Joseph Disease in the Australian Aboriginal Communities of Groote Eylandt and Yirrkala

This is a sad stuff. Those percentages are quite high.

>>Some 5% of inhabitants have this inherited disease.

I’m thinking that if that 5% breeds with 5% of the clean 95% that over a reasonably short period of time the percentage of people with the disease is going to balloon.

Peak Warming Man said:

>>Some 5% of inhabitants have this inherited disease.

I’m thinking that if that 5% breeds with 5% of the clean 95% that over a reasonably short period of time the percentage of people with the disease is going to balloon.

There are significant ethical issues around this topic. The disease can be tested genetically long before onset, and given there is no treatment there is not much value in the test except for helping people prepare their lives for the degeneration, and for them to make decisions on whether to have children.

Fellow in this story is a great-grandfather was not diagnosed until he was 63, he has dozens of descendants now.
http://www.abc.net.au/news/2014-04-21/prevalence-of-rare-brain-disease-increasing-in-nt-communities/5401948

Like Huntingtons. You are adult and have reproduced before you know you have it. Or this was the case until quite recently. I have been told by one of my patients about a family member of hers, where the family know it’s there in the genes, at least one (and I think it was more than one) of the affected people put their affairs in order and then went out and killed themselves before it got to the point of not being able to do so.

Fellow in this story is a great-grandfather was not diagnosed until he was 63, he has dozens of descendants now.
http://www.abc.net.au/news/2014-04-21/prevalence-of-rare-brain-disease-increasing-in-nt-communities/5401948

“The former Labor government promised $10 million to the foundation from mining royalties fund the Aboriginals Benefit Account.

The foundation had planned to invest the money in perpetuity to provide long-term funding and to establish permanent services in communities where MJD was growing. At present, it only provides part-time support.

New Indigenous Affairs Minister Nigel Scullion cancelled the grant in February, though the Government is offering the foundation $500,000 a year to cover the loss.”
——

Not as much as Barnaby spent doing up his office.

Groote Eylandt isn’t a mining town? I thought the population of mining towns was pretty well all transient. That’s true of Cooper Pedy and Olympic Dam, to cite two wildly differing examples.

mollwollfumble said:

Groote Eylandt isn’t a mining town? I thought the population of mining towns was pretty well all transient. That’s true of Cooper Pedy and Olympic Dam, to cite two wildly differing examples.

It isn’t a town at all. It is a large island, about 60 km across, with several towns. It has been inhabited for at least 3000 years. Its permanent population is about 1600, local language is Anindilyakwa.

There is a manganese mine with about 800 transient employees, but the statistics on MJD referred to above relate to the permanent population.

PermeateFree said:

Chat ————————>

PermeateFree said:

PermeateFree said:

Chat ————————>

Yeah, but I luffed.

Small dogs are not dogs.

sarahs mum said:

Fellow in this story is a great-grandfather was not diagnosed until he was 63, he has dozens of descendants now.
http://www.abc.net.au/news/2014-04-21/prevalence-of-rare-brain-disease-increasing-in-nt-communities/5401948

“The former Labor government promised $10 million to the foundation from mining royalties fund the Aboriginals Benefit Account.

The foundation had planned to invest the money in perpetuity to provide long-term funding and to establish permanent services in communities where MJD was growing. At present, it only provides part-time support.

New Indigenous Affairs Minister Nigel Scullion cancelled the grant in February, though the Government is offering the foundation $500,000 a year to cover the loss.”
——

Not as much as Barnaby spent doing up his office.

I think something should at the least be spent on genetic counselling. Then people might have some idea about the options for doing something about it.

I didn’t know until recently that one needed a permit to go to Groote Eylandt.

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

TATE says (as a matter of undisputed fact) that first Makassan/Australian contact was mid-1700’s:

https://en.wikipedia.org/wiki/Makassan_contact_with_Australia

I’d have thought there could be a good deal of doubt about it, but what do I know on such things?

Reading the whole TATE article, I note that it makes no mention of Machado-Joseph disease.

A job for dv there I think.

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

The Rev Dodgson said:

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

In fact they traded with the indigenous peoples for the rights to fish there.

roughbarked said:

The Rev Dodgson said:

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

In fact they traded with the indigenous peoples for the rights to fish there.

Seems like a reasonable arrangement.

The Rev Dodgson said:

roughbarked said:

The Rev Dodgson said:

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

In fact they traded with the indigenous peoples for the rights to fish there.

Seems like a reasonable arrangement.

Is the side-line of providing transport for undocumented one-way tourists the problem?

roughbarked said:

The Rev Dodgson said:

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

In fact they traded with the indigenous peoples for the rights to fish there.

Ref? How do we know this?

gsnnfng

Chance of showers and storms. To 36C. Big storm last night.. mostly missing here.

I pulled a muscle in my lower back while up a tree trimming branches on Saturday. Much pain, drugs.. sitting still.. feeling better now.

It’s not a place you hear much about. Apparently the mine there provides a quarter of the world’s supply of manganese.

Bubblecar said:

It’s not a place you hear much about. Apparently the mine there provides a quarter of the world’s supply of manganese.

I’ve got a couple of friends working up there, sounds like a decent place

The Rev Dodgson said:

Also, if Indonesians have been fishing those waters for at least 250 years, doesn’t that give them sea rights?

If not, why not?

Because there was no such country as “Indonesia” back then. Perhaps the sea rights should belong to the Netherlands?

More seriously, because of conflicting oceanic territorial claims and the law of the sea.

A rare autosomal dominantly inherited neurodegenerative disease that causes progressive cerebellar ataxia, which results in a lack of muscle control and coordination of the upper and lower extremities. The symptoms are caused by a genetic mutation.

Patients with severe forms of MJD have a life expectancy of approximately 35 years. Those with mild forms have a normal life expectancy. The cause of death of those who die early is often aspiration pneumonia.

—-

Nasty

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

Nah, there’s physical evidence going back to the 16th century.

dv said:

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

Nah, there’s physical evidence going back to the 16th century.

Also … no one on earth thinks James Cook got here first. Even among Europeans, his voyage was the 8th or 9th to explore Australia.

dv said:

dv said:

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

Nah, there’s physical evidence going back to the 16th century.

Also … no one on earth thinks James Cook got here first. Even among Europeans, his voyage was the 8th or 9th to explore Australia.

But we have documentary evidence of at least one on earth who does think JC got there first!

dv said:

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

Nah, there’s physical evidence going back to the 16th century.

And other evidence going back a further 1,000 years. The interaction between Aborigines and the Macassan trepangers goes back long before European contact, although earlier fleets may have been smaller. History does not begin with the Europeans, as some Englishmen would have you believe.

PermeateFree said:

dv said:

mollwollfumble said:

Iirc, the permit is a relatively recent thing, implemented at the same time as the Arnhem land one

> Makassan

Iirc, it was Flinders who first met the Makassans. He asked how long they had been coming to Austalia and found that it was relatively recent – James Cook got here first.

Nah, there’s physical evidence going back to the 16th century.

And other evidence going back a further 1,000 years. The interaction between Aborigines and the Macassan trepangers goes back long before European contact, although earlier fleets may have been smaller. History does not begin with the Europeans, as some Englishmen would have you believe.

IkR

dv said:

PermeateFree said:

dv said:

Nah, there’s physical evidence going back to the 16th century.

And other evidence going back a further 1,000 years. The interaction between Aborigines and the Macassan trepangers goes back long before European contact, although earlier fleets may have been smaller. History does not begin with the Europeans, as some Englishmen would have you believe.

IkR

Well you didn’t say so, but I was more concerned with Moll’s hearsay account that has taken on such importance.