When the cell digests itself: How inherited neurodegenerative diseases develop

A tangle of pockets, tubes and sac-like membrane structures runs through the cells of humans, animals, plants and fungi—the endoplasmic reticulum, or ER for short. In the ER, proteins are manufactured, folded into their three-dimensional structure and modified, lipids and hormones are produced and calcium concentrations in the cell are controlled. In addition, the ER forms the basis for the cellular transport system, feeds misfolded proteins to intracellular disposal and renders toxins that have entered the cell harmless.

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