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Abstract
Discrimination between properly folded proteins and those that do not reach this state is necessary for cells to achieve functionality. Eukaryotic cells have evolved several mechanisms to ensure secretory protein quality control, which allows efficiency and fidelity in protein production. Among the actors involved in such process, both endoplasmic reticulum (ER) and the Golgi complex play prominent roles in protein synthesis, biogenesis and secretion. ER and Golgi functions ensure that only properly folded proteins are allowed to flow through the secretory pathway while improperly folded proteins have to be eliminated to not impinge on cellular functions. Thus, complex quality control and degradation machineries are crucial to prevent the toxic accumulation of improperly folded proteins. However, in some instances, improperly folded proteins can escape the quality control systems thereby contributing to several human diseases. Herein, we summarize how the early secretory pathways copes with the accumulation of improperly folded proteins, and how insufficient handling can cause the development of several human diseases. Finally, we detail the genetic and pharmacologic approaches that could be used as potential therapeutic tools to treat these diseases.
Highlights
Secretory and transmembrane proteins are synthesized in the endoplasmic reticulum (ER) and transported to the Golgi compartment for additional maturation
ER-dependent protein quality control imposes a collaboration between protein synthesis and productive folding, ER-associated degradation (ERAD) [2] and ER homeostasis control [3]
Several environmental and chemical conditions can alter the functionality of the ER and Golgi complex causing the accumulation of improperly folded proteins, which impinge on cellular proteostasis
Summary
Secretory and transmembrane proteins are synthesized in the endoplasmic reticulum (ER) and transported to the Golgi compartment for additional maturation. To prevent immature proteins production and accumulation, mammalian cells developed different strategical quality checkpoints along the secretory pathway starting from their synthesis until their final destination. Cells 2019, 8, 1347 production and accumulation, mammalian cells developed different strategical quality checkpoints along the secretory pathway starting from their synthesis until their final destination These different checkpoints are important to prevent the aberrant secretion of improperly folded proteins, which could in turn become proteotoxic and of cause severalfolded human diseases (e.g., degenerative and important to prevent the aberrant secretion improperly proteins, which could in turn become proliferativeand disorders). [5]. human diseases (e.g., degenerative and proliferative disorders) [5]
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