BLUE PEOPLE OF KENTUCKY

We’re not talking about...• The smurfs• Clinically depressed people• Fans of B.B. King• The Blue Man Group• Avatarians

Ingesting elemental silver can also turn the skin blue. This man, Paul Karason, rubbed colloidal

silver on his face and skin many years ago to treat a skin condition, which made him slowly turn a

bluish/grey color.

WHO ARE THEY?"Blue People" are descendants of Martin Fugate, who suffered from a recessive gene which limits or stops the production of the enzyme diaphorase (enzymes that catalyze the reduction of various dyes to act as hydrogen acceptors NADH + NADPH).

What makes them blue?• Methemoglobinemia• Red blood cells (RBC) from blue people are

deficient in NADH diaphorase activity and cannot reduce the oxidized iron in methemoglobin (metHb).

• The lack of this enzyme gives people a blueish cast to their skin rather than pink. Lack of oxygen to RBC gives this color.

Interview

• '"He was almost purple,"' his father, Alva Stacy, recalls." Benjy was born in a maternity ward near Hazard, Kentucky and was rushed by ambulance to a medical clinic in Lexington to find help for his blue problem. Days of testing provided no answers; but then Benjy's grandmother told the doctor a story about the "blue" Fugates. Incredibly, Benjy had inherited a gene dating back over 162 years! Thankfully, Benjy lost his blue shade after a few weeks and the only lingering effects were blue fingernails and lips when he was cold or angry.

Fugate FamilyFather + 4 children are blue

Luna Fugate and John Stacey

• Lorenzo Dow Fugate and Eleanor Fugate. Lorenzo Dow was also know as "Blue Anze"

Real Folks

Of Lonesome Creek, Kentucky

In Hazard, the County Seat

Symptoms

• Blue colored skin• Headaches• Fatigue• Coma• Death

Treatment

• The homozygous patient (bb) daily ingestion of ascorbic acid to increase non- enzymatic reduction of methemoglobin

• or the use of methylene blue (oddly enough)- acts as an e- donor

Degree of Blueness

• BB for the normal allele – not blue• Bb - heterozygotes for the blue trait -

do contain some NADH diaphorase activity –(blue lips, blue fingernails)

• bb – homozygous recessive - blue skin

Blue Pedigree of Fugates

Get Handout • = male with it

• = female with it

Pedigree Symbols

More Pedigree Symbols

Found on WikiPedia: There is a mistake here. Can you find it?

bb Bb BB

Pedigree of Fugates

•Can you work out the genotypes?

Pedigree with Genotypes

• trait visible

• carrier

PRINT AND COLOR

Pedigree Videos• Media Showcase: Pedigree

• Understanding Pedigrees (6:41)• Solving a Pedigree - the Fast Way (example

1) (6:42)

• Solving a Pedigree The Fast Way -example 2(6:46)

You Will Make a Pedigree of Your Own Family

GET INSTRUCTION SHEET

Choose a trait from the lab of Variations of a Human Face (the one you drew the face on)