@article{THESIS, recid = {10150}, author = {Zhong, Frank Wang}, title = {Defining Assembly of a Translocon for Multipass Membrane Protein Biogenesis}, publisher = {University of Chicago}, school = {Ph.D.}, address = {2023-12}, number = {THESIS}, pages = {129}, abstract = {The cellular proteome is comprised of both soluble proteins and membrane proteins. Membrane proteins make up 30% of the human proteome and defects in their biogenesis results in a wide range of diseases. While biogenesis of membrane proteins with a singular transmembrane domain (TMD) is relatively well understood, the biogenesis of membrane proteins with multiple TMDs (“multipass”) is less clear. The TMDs of multipass membrane proteins are diverse, with varying hydrophobicities, and require folding within the lipid bilayer. To overcome this set of challenges, the Sec61 translocon has been shown to assemble various complexes during protein synthesis. Here, we show that for multipass membrane proteins, a Sec61 translocon specific for this class of membrane proteins exists and is assembled in response to defined signals. Furthermore, we show that two different novel accessory factors associate with the Sec61 translocon and are involved in protein biogenesis. We first define the existence of a “multipass translocon.” The multipass translocon is distinguished by three components that selectively bind the ribosome–Sec61 complex during multipass protein synthesis: the GET- and EMC-like (GEL), protein associated with translocon (PAT) and back of Sec61 (BOS) complexes. We show that assembly of the multipass translocon is in response to defined signals and depletion of multipass translocon components results in multipass membrane protein biogenesis and topogenesis defects both in vivo and in vitro. These results establish the mechanism by which nascent multipass proteins selectively recruit the multipass translocon to facilitate their biogenesis. In addition to this work, we characterize the role of two translocon accessory factors, RAMP4 and FKBP11, and their role in protein biogenesis. For both proteins, we demonstrate xi that they are involved in the synthesis of proteins with long lumenal loops. Specifically, for FKBP11, RNA sequencing of ribosome-translocon complexes implicates a bias for proteins that have long translocated lumenal domains. Analysis of insertion intermediates shows dynamics of FKBP11 association with the Sec61 translocon, with recruitment being concomitant with the oligosaccharyl transferase complex (OST). Depletion of FKBP11 further leads to membrane protein instability at the cellular level. In summation, this work defines the ER translocon as a dynamic assembly whose subunit composition adjusts co-translationally to accommodate the biosynthetic needs of its diverse range of substrates, from single-pass to multi-pass.}, url = {http://knowledge.uchicago.edu/record/10150}, doi = {https://doi.org/10.6082/uchicago.10150}, }