Official websites use .gov
A
.gov website belongs to an official
government organization in the United States.
Secure .gov websites use HTTPS
A lock (
) or https:// means you've safely
connected to the .gov website. Share sensitive
information only on official, secure websites.
A hypothetical model of selective neuronal vulnerability to NMD perturbations. During neuronal differentiation, NMD activity is suppressed by miRNAs to a minimal level required for viability. Additional insults to NMD, either by genetic mutations in NMD factor genes (early onset, e.g. UPF3B) or by the accumulation of NMD-inactivating molecules (late onset, e.g. R-DPR), would further reduce the activity below the required threshold, leading to pathological outcomes.
