Blue Fugates

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 methemoglobin into hemoglobin in red blood cells). The absence of this enzyme produced a disproportionate amount of methemoglobin in the blood, tinting it blue. Because each was only a carrier, neither Martin nor his wife suffered from the condition. However, by Mendelian inheritance, 25% of their children would be homozygous recessive and suffer from the condition, while 50% of their children would be asymptomatic heterozygous carriers like their parents.

Despite their bluish tint, the Fugates did not have any of the numerous sequelae associated with sever methemoglobinemia. As of 1982 only two or three members of the family had the condition (Carnegie 1997, p. 214). [Carnegie Library of Pittsburgh. Science and Technology Dept. "The Handy Science Answer Book: 2nd ed". Detroit, Michigan: Visible Ink Press, 1997. p. 214]

External links

*"Straight Dope" [http://www.straightdope.com/classics/a980724.html article on the Fugates of Appalachia, an extended family of blue-skinned people]
* [http://www.rootsweb.com/~kyperry3/Blue_Fugates_Troublesome_Creek.html The Blue People of Troublesome Creek]
* [http://www.geocities.com/luvacuzn6/BlueFugates.html Genealogical history of the Fugates]
* [http://www.nclark.net/BluePeopleofTroublesomeCreek.html]

Notes