RIDD‐mediated degradation of BLOC1S1 mRNA can promote the degradation of Huntington's protein, thereby alleviating the progression of Huntington's disease.[46] Therefore, restoring TDP‐43 nuclear function rescues autolysosome deficits in neurodegeneration, BLOC1S1's ability to modulate TDP‐43 shuttling positions it as a potential therapeutic node for both bacterial infections and TDP‐43‐mediated neuropathologies. Here, TARDBP is linked to juvenile Huntington disease.