Protein transport protein Sec61 subunit alpha isoform 1

Definition:

Component of SEC61 channel-forming translocon complex that mediates transport of signal peptide-containing precursor polypeptides across the endoplasmic reticulum (ER). Forms a ribosome receptor and a gated pore in the ER membrane, both functions required for cotranslational translocation of nascent polypeptides. May cooperate with auxiliary protein SEC62, SEC63 and HSPA5/BiP to enable post-translational transport of small presecretory proteins. Component of a ribosome-associated ER translocon complex involved in multi-pass membrane protein transport into the ER membrane and biogenesis. The SEC61 channel cooperates with the translocating protein TRAM1 to import nascent proteins into the ER. Controls the passive efflux of calcium ions from the ER lumen to the cytosol through SEC61 channel, contributing to the maintenance of cellular calcium homeostasis. Plays a critical role in nephrogenesis, specifically at pronephros stage (By similarity).

References:

[1]. Philip T McGilvray, et al. An ER translocon for multi-pass membrane protein biogenesis. Elife. 2020 Aug 21;9:e56889. [Content Brief]

[2]. Patrick J Chitwood, et al. An intramembrane chaperone complex facilitates membrane protein biogenesis. Nature. 2020 Aug;584(7822):630-634. [Content Brief]

[3]. H Do, et al. The cotranslational integration of membrane proteins into the phospholipid bilayer is a multistep process. Cell. 1996 May 3;85(3):369-78. [Content Brief]

[4]. Desirée Schubert, et al. Plasma cell deficiency in human subjects with heterozygous mutations in Sec61 translocon alpha 1 subunit (SEC61A1). J Allergy Clin Immunol. 2018 Apr;141(4):1427-1438. [Content Brief]

[5]. Sven Lang, et al. Different effects of Sec61α, Sec62 and Sec63 depletion on transport of polypeptides into the endoplasmic reticulum of mammalian cells. J Cell Sci. 2012 Apr 15;125(Pt 8):1958-69. [Content Brief]

[6]. Sarah Haßdenteufel, et al. Chaperone-Mediated Sec61 Channel Gating during ER Import of Small Precursor Proteins Overcomes Sec61 Inhibitor-Reinforced Energy Barrier. Cell Rep. 2018 May 1;23(5):1373-1386. [Content Brief]

[7]. Suzanna L Meacock, et al. Different transmembrane domains associate with distinct endoplasmic reticulum components during membrane integration of a polytopic protein. Mol Biol Cell. 2002 Dec;13(12):4114-29. [Content Brief]

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