New research throws fresh insights into neurodegenerative diseases

Scientists from The Scripps Research Institute have solved a long-standing mystery of how cells conduct "quality control" to eliminate the toxic effects of a certain kind of error in protein production. The findings may lead to a better understanding of a host of neurodegenerative diseases.

The research was published in an advance, online issue of the journal Nature on September 12, 2010.

"It is exciting because we are dealing not only with a process that is clearly relevant for physiology and disease," said Scripps Research Assistant Professor Claudio Joazeiro, who led the study, "but also with new biology."

The new study suggests how cells in eukaryotic organisms, like humans, sense and destroy "non-stop" proteins that remain stuck in the ribosome, the protein manufacturing plant of the cell.

Proteins "R" us
It's hard to overemphasise the importance of proteins in the body, as they participate in virtually every cellular process. Proteins are the end result of one of the central tenets of biology (known as the "central dogma"), which tells us that DNA is used to make RNA, which, in turn, is used to make proteins. In healthy cells, the ribosome translates the code carried by a messenger RNA (mRNA) to link together protein building blocks (amino acids) in a particular order to form specific proteins.

But errors happen - which is why the body has a host of different quality control mechanisms to ensure that the proteins that emerge from this process are flawless.