Blue Fugates
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The Blue Fugates are an extended family from the Appalachian Mountains famous for having a high occurrence of the rare disorder methemoglobinemia. Martin Fugate, a French immigrant and patriarch of the family, settled near Hazard, Kentucky circa 1800. He and his wife were carriers of the recessive methemoglobinemia (met-H) gene, which limited and/or stopped the body's production of the enzyme diaphorase (which breaks down methemologbin into hemoglobin in red blood cells). The absence of this enzyme produced a disproportionate amount of methemoglobin in the blood, tinting it blue.
Despite the bluish tint, there are no known health hazards associated with methemoglobinemia. Fugate's family suffered from the condition because of excessive inbreeding. When both spouses had the recessive gene, their child would be blue. As the family became mobile following World War II and began to move outside of their Kentucky valley, the inbreeding ceased. As of 1982 only two or three members of the family had the condition (Carnegie 1997, p. 214).[1]
[edit] External links
- Straight Dope article on the Fugates of Appalachia, an extended family of blue-skinned people
- The Blue People of Troublesome Creek
- Genealogical history of the Fugates
- [1]
[edit] Notes
- ^ Carnegie Library of Pittsburgh. Science and Technology Dept. The Handy Science Answer Book: 2nd ed. Detroit, Michigan: Visible Ink Press, 1997. p. 214
