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The Secretory Capacity of a Cell Depends on the Efficiency of Endoplasmic Reticulum-Associated Degradation

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Dislocation and Degradation of Proteins from the Endoplasmic Reticulum

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 300))

Abstract

Plasma cells, like other “professional” secretory cells, are capable of secreting thousands of proteins per second. To accomplish this impressive task, they contain a highly developed endoplasmic reticulum (ER), where newly synthesized proteins must fold and assemble to native structures before secretion. Protein biogenesis in the ER is coupled to a tight quality control schedule: aberrant molecules produced upon failure of the folding/oligomerization processes are retained in the ER, and eventually degraded by ER-associated degradation (ERAD) pathways. The activity of the ERAD machinery therefore needs to be adapted to variations in the load of the ERwith cargo proteins. If ERAD is insufficient, misfolded proteins accumulate causing ER stress, apoptosis, and ER storage diseases. The capacity of ERAD also critically determines the efficiency of protein secretion. Here we summarize recent findings highlighting the role of ERAD in disease and development, particularly in professional secretory cells.

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Author information

Authors and Affiliations

  1. Institute for Research in Biomedicine, Via V. Vela 6, 6500, Bellinzona, Switzerland

    M. Molinari

  2. Università Vita-Salute SanRaffaele, DiBiT, Via Olgettina 58, 20132, Milan, Italy

    R. Sitia

Authors
  1. M. Molinari
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  2. R. Sitia
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Editors and Affiliations

  1. Department of Medical Microbiology, Leiden University Medical Center, Albinusdreef 2, 2333, ZA, Leiden, The Netherlands

    Emmanuel Wiertz Ph. D.  & Marjolein Kikkert Ph. D.  & 

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Molinari, M., Sitia, R. (2006). The Secretory Capacity of a Cell Depends on the Efficiency of Endoplasmic Reticulum-Associated Degradation. In: Wiertz, E., Kikkert, M. (eds) Dislocation and Degradation of Proteins from the Endoplasmic Reticulum. Current Topics in Microbiology and Immunology, vol 300. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-28007-3_1

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