Article
Reversible aggregation of PABPN1 pre-inclusion structures.
Nucleus (Austin, Tex.) - 1 Jan 2000
Raz Vered, Abraham Tsion, van Zwet Erik W, Dirks Roeland W, Tanke Hans J, van der Maarel Silvère M
Abstract excerpt
Increased aggregation of misfolded proteins is associated with aging, and characterizes a number of neurodegenerative disorders caused by homopolymeric amino acid expansion mutations. PABPN1 is an aggregation-prone nuclear protein. Natural aggregation of wild-type (WT) PABPN1 is not known to be disease-associated, but alanine-expanded PABPN1 (expPABPN1) accumulates in insoluble intranuclear inclusions in muscle...
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