Naked Science Forum
Life Sciences => Physiology & Medicine => Topic started by: tamster on 02/12/2004 07:13:24
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not too terribly fun diseases.
anyone know about new research on these two diseases? is there anything to stop the body from attacking the myelin sheath of the spinal column? once paralyzation has set in, if the sheath can be repaired, will the paralyzation leave?
enquiring minds want to know.
the tamster
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Lots of variations on severity. I think X linked genetic metabloic disease, but not always X linked because both sexes can have ALD, I'm sure. These orphan diseases are tough because they're so rare that funding to research them is scarce. As far as ALD, there's always Lorenzo's oil, but it doens't work in all cases. I think there's some research going on for other medications, and I understand there was some talk of stem cell research in that area as well. You're right. Definitely not fun diseases.
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well, the women carry it, and there is a 50-50 chance of her sons being affected by it.
lorenzo's oil is a good thing.
tams
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ALD is an X-linked recessive disease, so only boys get it, as girls have a dominant gene on the second X chromosome. The only way girls could get it would be for their father to have reproduced while they were still able, and for their mother to be a carrier. from ehat little I know of the disease thats pretty unlikely, although maybe there are other non-x-linked forms out there.
It is caused by a breakdown of the myelin that surrounds the nerve cells, which results in slowing down of the nerve impluses until eventually the signals never reach the destination. This has an effect on the adrenal gland and prevents it functioning to break down very long chain fatty acids (VLCFA's) correctly, so they accumulate. this is what causes the symptoms, and this is what Lorenzo's oil was effective in treating. It reduced the levels of the VLCFA's. However, Lorenzo's oil has no effect on neurological degeneration, which is why Lorenzo didn't recover.
Augusto Odone, Lorenzo's father, cofounded the Mylein Project: http://www.myelinproject.co.uk also http://www.myelin.org for info. There are several treatments being worked on, the most interesting is based on transplantation of Schwann cells, possibly from bone marrow (stem cells) then treatment with beta-interferon to induce remyelination.
I'm guessing that if the myelin sheath could be repaired, then the adrenal gland problems would disappear, and the patient would completely recover? sounds promising anyway.
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really !
i was told that my brother's daughter will be a carrier. hmmm...
now, i'm confused. ( okay, i'm blonde, it's an easy thing to do )
yep, the lorenzo's oil has kept my two nephews completely asymptomatic. thank goodness.
i wonder if they know about the schwann cells at oregon health university. that's the first i've ever heard of it.
before this hit my brother, he was a black belt in karate, and a carpenter. hell of a thing.
thanks.
tams