"Mike" <[EMAIL PROTECTED]> writes:
Hi Folks,
Ron wrote:
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> Mike: I am now among the very special people who have visited your
> referenced website, thank you. As you know, there are inherited and
> acquired forms of mitochondrial disease and we certainly do not deny the
> existence of this disorder.
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You're welcome, Ron. Thanks for taking the time to visit. I would be
interested in your info on acquired mito disorders. To date, all of the
research that we have access to seems to indicate that all mito disorders
are inherited. Would you mind privately emailing me some references?
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> However, to attempt to explain away the death of
> ten children in one child abuser's family
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I wasn't aware that the abuse charges were substantiated, just that there
was suspicion of abuse. I wasn't trying to explain the deaths away, just
offering an alternative to the straight murder theory.
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> all of whom started out their
> childhood normal,
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Not unusual in mito disorders, like I said in an earlier post, the symptoms
can appear anywhere from a few days to the age of 11 or 12, and the child
can appear "normal" and healthy before the onset of symptoms. It's also
quite possible that some symptoms and clinical signs were overlooked back
then. Even in the 80's, it took 6 years before my wife was properly
diagnosed with the MELAS Syndrome, a mito disorder.
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> and neither parent obviously died of this disease,
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Neither parent has to die, mito disorders don't always kill, though most do
eventually. Both parents don't need to have a mito disorder to pass it on
to their children, either (see below).
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> by
> resorting to a hereditary disease ( which can never be proven) as the
> cause of these deaths is really a s-t-r-e-t-c-h.
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Can be proven in the mother's case with successful testing, and in the
event of a definite diagnosis, suspected in the children's.
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> Is Noe a born again Christian?
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What the heck does *this* have to do with the deaths of the children? I
believe from what I've read, that the father and mother are (or were?)
devote Catholics.
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> What kind of God would want a family to keep having children that would
> inherit a lethal anomaly?
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Ain't God's fault, can't blame Him for mistakes made in His name by
mankind.
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> Mike, is the mode of inheritance for the
> inherited form of mitochondrial disease known?
<snip>
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Depends on the mito disorder. The defects that can cause a mito disorder
can occur in either of two "locations". Either in the nuclear DNA, when
the communication between the cell and the mitochondria breaks down, and
perfectly functional mitochondria don't do their job of converting blood
sugar into ATP (the cell's energy "fuel"); or in the mitochondrial DNA, and
the Krebs (or citric acid) cycle doesn't work properly and not enough blood
sugar gets converted into ATP. In either case, the result is the same;
cells starve and die. Depending on where in the body the defect is
located, you'll see different symptoms and mortality rates.
In the case of nuclear DNA, the inheritance factor is as in any other
inheritance pattern. In the case of mitochondrial DNA (mtDNA), however, it
is a completely different story. We inherit ALL of our mtDNA from our
mothers. So while a mother can pass the disorder along to both her male
and female children, only her daughters can pass it along to their
children.
The inheritance isn't consistent, some children can inherit a severe mito
disorder, and some a much milder case. In some families (as in a number of
the families in our support group) all of the children can be severely
affected by the disorder. The most surviving children significantly
affected in one family, that I know of, is 5.
Actually, I just want to see the truth come out in the Noe's case. Whether
that leads to murder *or* a medical explanation, or even both. The support
that I offered to the folks in Philly, came after I heard of the interest
in the case by Dr. Robert Naviaux (founder of the MMDC at UCSD, and also
assistant professor of internal medicine at UCSD) and Angie Longenecker
(clinical nurse coordinator), both of the Mitochondrial and Metabolic
Disease Center at the University of California, San Diego.
I'd hate to see someone convicted of a crime just because folks *think*
that they did it.
"When you hear the sounds of galloping hooves, think of horses, not
zebras!"
Not if you live in Africa... <grin>
TTFN
Mike <[EMAIL PROTECTED]>
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