break the rules - 2 Laugh to death

In the early 1950's, New Guinea witnessed an epidemic. It was called 'Kuru' - laughing death. The individuals suffering from this disease laughed for no apparent reasons. Such a laughter indicated death.
In New Guinea, the Fore tribe were severely affected. So much so that their tribe was driven close to extinction because of this disease KURU which literally means 'trembling with fear'. It is a neurological disease. Other than untimely laughter, the individuals suffered from loss of muscular coordination, tremor, difficulty in walking, loss of memory and difficulty to swallow at various stages of the disease. It is fatal and one dies within 3 months to 2 years of onset of the disease. The infection may reside without any symptoms for 2 years to 30 years.

It was first suggested that this was a genetic disorder and when it was found to be infective, this was ruled out. But, the agent of infection remained mysterious.
By studying the symptoms, it was believed that the agent was a slow virus. This was proved incorrect when the real culprit emerged. The disease was caused by prions. The idea of a protein being an infectious agent was not something, many of the scientists studying the disease, thought of.

One of the customs of Fore tribe was that they believed in paying respect to their dead by consuming their corpses. This cannibalistic ritual lead to the spread of prions very easily and effectively.

When they finally gave up their unique way of showing love and respect for their elders, the spread of the disease was controlled and the disease was eradicated.

Stanley Prusiner, who studied prions and described its molecular biology received the Nobel in the year 1997.

break the rules-1

If there is anything that fascinates thats science. Especially, the human body. How complex is this machine????? It has been more than 10,000 years (since we started living a life that is different from other animals on the planet), still we are no where close to proclaim that our understanding of the magnificently complex 'human body' is complete.

Nature had always eluded human comprehension.

Discovery of prions toppled the perception that infectious agents are viruses or any living organisms. Prions are proteins that are capable of causing infections on their own.

Prions are thought to be misfolded proteins that once inside a cell, could cause the normal proteins to misfold and prevent the cell from performing their function.
The famous Madcow disease and Scrapie are caused by prions.

You can expect more posts on prions in the following weeks.

bye.