Prion-like Proteins Cause Disease | multisystem proteinopathy and ALS The Scientist Magazine®

Individuals with a rare inherited syndrome called multisystem proteinopathy (MSP) harbor misbehaving proteins that fold incorrectly, change the shape of surrounding proteins, and clump together—much the way disease-causing prions do. The results, published today in Nature, suggest that the 250 or so human proteins with similar prion-like domains may also be involved in diseases of the brain or other organs.

H. J. Kim et al., “Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS,” Nature, doi:10.1038/nature11922, 2013.

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