The Prado Museum in Madrid hangs a pair of paintings by a seventeenth century painter Juan Carreno de Miranda, paintings of a grossly fat monstrous but innocent five year old girl named Eugenia Martinez Vallejo, called 'La Monstrua vestida' and 'La Monstrua Desnuda': The Monster Clothed and The Monster Naked. They both show an obese five year old girl whose tiny hands and feet and strange shaped eyes and mouth, kinda a suitable freak for a circus. It is plain and obvious that Eugenia showed us symptoms of a rare inherited disease called Prader Willi syndrome. Prader-Willi syndrome children are born floppy, pale skinned, refuse to suck at the breast but eat everything omnivorously, never satisfied at food, and so become grossly obese. Their sex organs are also undevelopped and yet, they are mentally retarded. Unfotunately, like autistic kids they often throw tantrums especially about food.
A family whose descendant has this syndrome usually has an opposite syndrome crop up in their same family tree, called Angelman's syndrome. Founded by Harry Angelman, a doctor from Lancashire called the patients are 'puppet children'. Opposing the Prader Willi symptoms, the children are taut, hyperactive, insomniac, small headed, long jawed and often to stick out their tongues like happy dogs. Their favorite hobbies are to play and laugh but never learn to speak and so severelly retarded.
It soon found that the same chunk of chromosome 15 is missing in the both patients. But the difference is that in Prader Willi syndrom, the missing chunk was from the father's chromosome, while in Angelman's syndrome the missing chunk was from the father's.
Prader Willi syndrome is transmitted through men and vice versa. In summary, we can conclude from both syndromes that, gene remembers which parent it came from because they have paternal or maternal imprint. Thus, the body expresses only the gene that switches on, not both.
Labels: angelman, biology, children, doctor, genes, genetic chromosomes, genome, prader-willi, syndrome