All posts by Amber Dance

Paper Alert: Could Antisense Therapy One Day Squelch Toxic Repeats in ALS or FTD?

If toxic RNAs are a problem, then antisense oligonucleotides (ASOs) appear to be a solution. In this case, the toxic RNAs are transcribed from hexanucleotide repeats in the C9ORF72 gene, which can cause both amyotrophic lateral sclerosis and frontotemporal dementia in people (see Sep 2011 news). Transgenic mouse models expressing these repeats develop the same […]

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C9ORF72 Dipeptide Repeat Not Enough for Motor Neuron Disease

When expressed in mouse brain, the poly(GA) dipeptide repeat , which is translated from the C9ORF72 hexanucleotide expansion RNA, interfered with protein degradation and nucleocytoplasmic transport, report senior author Leonard Petrucelli and colleagues at the Mayo Clinic in Jacksonville, Florida. Poly(GA) aggregates sequestered HR23 proteins, which transport nuclear proteins to the proteasome for degradation.

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A152T: A Different Kind of Tau Mutation

Scientists debuted two new tauopathy models recently, and they are unlike other tau mice. Developed in parallel by two laboratories, these mice point to excitotoxicity as a possible mechanism by which the alanine-152-threonine tau mutation boosts risk for various neurodegenerative diseases. Tau aggregation was scant in the mice, which nonetheless exhibited age-related neuroinflammation, neurodegeneration, and […]

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