Adult Progeria (Werner's Syndrome)

  • Form of Progeria that has a later onset.
  • Autosomal Recessive mutation in the WRN gene which codes for DNA helicase.
  • Mutation leads to inability to repair DNA damage and replication defects resulting in the accumulation of damage and premature cellular aging.
  • Develop disorders of aging early in life such as: Cataracts, Type 2 Diabetes, Gray hair and Hair loss, Osteoporosis, atherosclerosis, and cancers.
  • Premature death from cardiovascular disease or cancer.

Table comparing Progeria vs Werner Syndrome

 

In the movie "Jack", Robbin Williams plays a 10-year-old boy with adult progeria:
 

"Robbin Williams stars as a mischievous 10-year-old who acts like any other boy his age, except he's in a grown-up's body."  

Watch the trailor for the movie "Jack" below and notice that they make a minor mistake.  The doctor says that "His cells are developing at 4 times the normal rate" but he should have said that his cells are aging at a faster rate!

 

Quote from Jack's final Graduation speech:

"But I say to you, "Hey, look at me." Please, don't worry so much, 'cause in the end none of us have very long on this earth. Life is fleeting. And if you're ever distressed, cast your eyes to the summer sky, when the stars are strung across the velvety night, and when a shooting star streaks through the blackness turning night into day -- make a wish think of me. And make your life spectacular. I know I did."  - Jack

Comments  

#1 Marcela G 2017-01-20 22:36
Thank for that Dr. You're awesome... have a nice day.
Quote
Category: Genetics Mnemonics

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