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Updated: Nov 3, 2025

Studying Pre-formed Fibril Induced α-Synuclein Accumulation in Primary Embryonic Mouse Midbrain Dopamine Neurons
Published on: August 16, 2020
Neuronal intranuclear inclusion disease: Polyglycine protein is the culprit
Michelle E Ehrlich1, Lisa M Ellerby2
1Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.
Abstract:
In this issue of Neuron, Boivin et al. (2021) show that a polyglycine-expanded protein, uN2CpolyG, is translated from an expansion of GGC repeats in the 5' UTR of the NOTCH2NLC (Notch homolog 2 N-terminal-like C) gene, defining a new pathological mechanism for neuronal intranuclear inclusion diseases (NIID).
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