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  An Update on Sec61 Channel Functions, Mechanisms, and Related Diseases

Lang, S., Pfeffer, S., Lee, P.-H., Cavalie, A., Helms, V., Forster, F., et al. (2017). An Update on Sec61 Channel Functions, Mechanisms, and Related Diseases. Frontiers in Physiology, 8: 887. doi:10.3389/fphys.2017.00887.

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© 2017 Lang, Pfeffer, Lee, Cavalié, Helms, Förster and Zimmermann. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY).
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 Creators:
Lang, Sven1, Author
Pfeffer, Stefan2, Author              
Lee, Po-Hsien1, Author
Cavalie, Adolfo1, Author
Helms, Volkhard1, Author
Forster, Friedrich1, Author
Zimmermann, Richard1, Author
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1external, ou_persistent22              
2Baumeister, Wolfgang / Molecular Structural Biology, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565142              

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Free keywords: ENDOPLASMIC-RETICULUM MEMBRANE; SIGNAL RECOGNITION PARTICLE; TAIL-ANCHORED PROTEINS; POLYCYSTIC LIVER-DISEASE; RIBOSOME-TRANSLOCON COMPLEX; MARINESCO-SJOGREN-SYNDROME; QUALITY-CONTROL PATHWAY; XBP1U MESSENGER-RNA; CA2+ LEAK CHANNELS; ER MEMBRANEPhysiology; ATP import; BiP; calcium leakage; endoplasmic reticulum; protein biogenesis; Sec61 complex;
 Abstract: The membrane of the endoplasmic reticulum (ER) of nucleated human cells harbors the protein translocon, which facilitates membrane integration or translocation of almost every newly synthesized polypeptide targeted to organelles of the endo- and exocytotic pathway. The translocon comprises the polypeptide-conducting Sec61 channel and several additional proteins and complexes that are permanently or transiently associated with the heterotrimeric Sec61 complex. This ensemble of proteins facilitates ER targeting of precursor polypeptides, modification of precursor polypeptides in transit through the Sec61 complex, and Sec61 channel gating, i.e., dynamic regulation of the pore forming subunit to mediate precursor transport and calcium efflux. Recently, cryoelectron tomography of translocons in native ER membrane vesicles, derived from human cell lines or patient fibroblasts, and even intact cells has given unprecedented insights into the architecture and dynamics of the native translocon and the Sec61 channel. These structural data are discussed in light of different Sec61 channel activities including ribosome receptor function, membrane insertion, and translocation of newly synthesized polypeptides as well as the putative physiological roles of the Sec61 channel as a passive ER calcium leak channel. Furthermore, the structural insights into the Sec61 channel are incorporated into an overview and update on Sec61 channel-related diseases-the Sec61 channelopathies- and novel therapeutic concepts for their treatment.

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Language(s): eng - English
 Dates: 2017-11-01
 Publication Status: Published online
 Pages: 22
 Publishing info: -
 Table of Contents: -
 Rev. Type: -
 Identifiers: ISI: 000414135400001
DOI: 10.3389/fphys.2017.00887
 Degree: -

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Title: Frontiers in Physiology
  Other : Front. Physiol.
  Abbreviation : FPHYS
Source Genre: Journal
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Publ. Info: Lausanne : Frontiers Research Foundation
Pages: - Volume / Issue: 8 Sequence Number: 887 Start / End Page: - Identifier: ISSN: 1664-042X
CoNE: https://pure.mpg.de/cone/journals/resource/1664-042X