My last research job was studying ALS. We had mouse models of the disease and were exploring how breeding two different strains of mice (many lab mice are inbred until they are
genetically identical
) might lead us to a gene that could be manipulated by drugs to help treat the disease onset and/or progression (diagnosis usually occurs after age 50, when people start noticing muscle weakness in extremities and normally die within 6 months to 5 years, depending on the severity of the mutation in the main gene causing it, or the length of time before they come in before diagnosis. By the time muscle weakness is noticed, the disease has significantly ravaged the motor neurons in the brain).
By mating two different strains of mice, we introduced slight genetic variations of the same genes that the diseased mice normally had, and discovered that it delayed the onset and progression of the disease. Unfortunately, the effect was too small, and the genome portions too large to cost-effectively narrow down and generate a possible therapeutic drug from that might've only helped a small subset of humans for a short time. It was stimulating research though, I learned a lot about this devastating disease, and met several patients and families touched by it in the process. It's not a way I'd want to go. Hawking doesn't have a traditional, typical form of ALS, but what he's accomplished with the obvious physical limitations is beyond impressive regardless.
But like his mind, Hawking's illness seems to be singular. Most patients with ALS—also known as Lou Gehrig's disease, for the famous baseball player who succumbed to the disease—are diagnosed after the age of 50 and die within five years of their diagnosis. Hawking's condition was first diagnosed when he was 21, and he was not expected to see his 25th birthday.
Why has Hawking lived so long with this malady when so many other people die so soon after diagnosis? We spoke with Leo McCluskey, an associate professor of neurology and medical director of the ALS Center at the University of Pennsylvania, to find out more about the disease and why it has spared Hawking and his amazing brain.
It would be hard to find a geek who doesn’t recognize the world-famous theoretical physicist Stephen Hawking, who turns 70 today, or his equally famous voice.
Being forced to use a speech synthesizer — as Hawking has since 1986 due to complications from his disease — would have left hindered most peoples’ ability to communicate. But somehow the computerized voice, and the length of time it takes him to type in his thoughts, lends even more weight to Hawking’s words. Below, we have collected audio of some of his best quotes on topics such as physics, space travel, and the meaning of life, all spoken in his iconic digital voice.
"Now, more than ever, the illusions of division threaten our very existence. We all know the truth: more connects us than separates us. But in times of crisis the wise build bridges, while the foolish build barriers. We must find a way to look after one another as if we were one single tribe.” -King T'Challa, Black Panther
The truth is incontrovertible. Malice may attack it. ignorance may deride it, but in the end, there it is. ~Winston Churchill
