New misfolding mechanism spotted

Study reports a generalized means of upsetting RNA translation, causing misfolded proteins -- and, possibly, neurodegeneration

Charles Choi
Aug 13, 2006
Scientists appear to have discovered a new general mechanism for generating misfolding proteins, perhaps underlying some cases of neurodegeneration, according to a new online report in Nature.When it comes to diseases involving misfolded proteins, scientists typically link a particular disorder with a specific misfolded protein, such as a prion or beta-amyloid, said coauthor Susan Ackerman at the Jackson Laboratory in Bar Harbor, Maine. This study, in contrast, found a more generalized mechanism that upsets the accuracy of RNA translation, creating misfolded proteins."Here we're talking about a mechanism that's responsible for many, many different proteins misfolding," she told The Scientist. "This is the first example of this mechanism at the mammalian level, and I doubt it's the only one."Ackerman and her colleagues focused on the sticky mutation in mice, which leads to tremors and ataxia due to loss of Purkinje cells in the cerebellum. The researchers found many...

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