-
Article
Open AccessPathologic tau conformer ensembles induce dynamic, liquid-liquid phase separation events at the nuclear envelope
The microtubule-associated protein tau forms aggregates in different neurodegenerative diseases called tauopathies. Prior work has shown that a single P301L mutation in tau gene, MAPT, can promote alternative tau...
-
Article
Correction to: Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT‑P301L mutation
In the original publication, Table 1 column heads are incorrectly formatted and aligned. The corrected Table 1 is given here.
-
Article
Open AccessQuaternary Structure Changes for PrPSc Predate PrPC Downregulation and Neuronal Death During Progression of Experimental Scrapie Disease
Prion diseases are fatal neurodegenerative diseases in mammals with the unique characteristics of misfolding and aggregation of the cellular prion protein (PrPC) to the scrapie prion (PrPSc). Although neuroinflam...
-
Article
Open AccessDiverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT-P301L mutation
Tau protein accumulation is a common denominator of major dementias, but this process is inhomogeneous, even when triggered by the same germline mutation. We considered stochastic misfolding of human tau confo...
-
Article
Open AccessProteasomal Inhibition Redirects the PrP-Like Shadoo Protein to the Nucleus
The Shadoo protein (Sho) exhibits homology to the hydrophobic region of the cellular isoform of prion protein (PrPC). As prion-infected brains gradually accumulate infectivity-associated isoforms of prion protein...
-
Article
Open AccessThe CNS in inbred transgenic models of 4-repeat Tauopathy develops consistent tau seeding capacity yet focal and diverse patterns of protein deposition
MAPT mutations cause neurodegenerative diseases such as frontotemporal dementia but, strikingly, patients with the same mutation may have different clinical phenotypes.
-
Article
Open AccessRole of p73 in Alzheimer disease: lack of association in mouse models or in human cohorts
P73 belongs to the p53 family of cell survival regulators with the corresponding locus Trp73 producing the N-terminally distinct isoforms, TAp73 and DeltaNp73. Recently, two studies have implicated the murine Trp...
-
Chapter
The PrP-Like Proteins Shadoo and Doppel
An almost unique place within protein databases, twenty-five years of study has underscored the enigmatically subtle role of PrPC in normal cell biology. It seems that PrP has evolved (and survived) to perform a ...
-
Chapter
Familial Prion Diseases Modeled in Cell Culture
The central pathogenic event in prion diseases is the posttranslational conversion of PrPC, a normal cell-surface glycoprotein, into PrPSc, the principal constituent of infectious prion particles (reviewed by Pru...