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The Scientist: NewsBlog:
Feudin? genetics style
[Entry posted at 9th April 2007 04:28 PM GMT]
| A nice AP story talks about the hypothesis that much of the fuel for the famed Hatfield-McCoy feud might be attributed to Von Hippel-Lindau disease, a rare genetic disorder that predisposes those affected to highly-vascularized tumors. The reasoning is that adrenal tumors might be responsible for many of the McCoys' notorious tempers. There are some great quotes from actual family members in the article.
I always liked reading and writing about Von Hippel-Lindau. The VHL tumor suppressor deactivates angiogenesis in an interesting oxygen sensing pathway. Normally VHL signals the degradation of hypoxia inducible factor HIF1alpha. In hypoxic conditions, or in the presence of a deactivating VHL mutation HIF1alpha and other protein players initiate transcription of pro angiogenic genes.
A book I recently reviewed for the magazine had another take on the famous Appalachian feud. David Sloan Wilson's Evolution for Everyone mentions research which reasons that a code of behavior adopted long ago by the Scots-Irish who moved to southern and eastern mountain regions of the United States held a sense of family honor very highly. Members of a family or group would risk life and limb to protect the reputation of the group. Research has shown that people from this background are more prone to aggression in certain experimental situations, although it's hardly a perfect science.
VHL might play a role in the violence, but I wouldn't want to start making generalizations. Hatfields, McCoys, and many others from that area of the country have suffered their fare share of sweeping statements. As Marilynn Marchione from the AP writes, 'Unfortunately for Appalachia, the feud is one of its greatest sources of fame.' Even more unfortunate is another oft-cited source of fame that doesn't bode well in terms of human genetic disease, inbreeding.
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The Problem is Pheos, not VHL by Joyce Graff [Comment posted 2007-04-15 13:14:13] The McCoy article suggests a link between the family feud and von Hippel-Lindau disease (VHL).
The real problem is pheochromocytoma, not VHL. Pheos are a tumor of the adrenal gland as described in the article. They occur in only 20% of people with VHL, and more often in some families than others. The hormone surges they produce are most often perceived as panic attacks or palpitations, not rage.
Pheos occur in the general population, and in people with any of six other genetic flaws. If anyone feels they are having uncontrolled high blood pressure, palpitations, unexplained bursts of panic or rage, and/or excessive sweating, they should ask their doctor to do a test called "plasma free metanephrines". This is the most accurate test for a pheo. Other tests only find 60-80% of pheos. They are not easy to diagnose.
Still today half of all pheos are diagnosed on autopsy. They are very dangerous. The good news is that once they are diagnosed they are almost always treated successfully.
And no matter how lousy we feel, we are all still responsible for our actions.
We wish you the best of health.
Joyce Graff, Executive Director
VHL Family Alliance
www.vhl.org 800-767-4845
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