eprintid: 10049829 rev_number: 19 eprint_status: archive userid: 608 dir: disk0/10/04/98/29 datestamp: 2018-06-11 15:24:40 lastmod: 2021-10-05 00:34:58 status_changed: 2018-06-11 15:24:40 type: article metadata_visibility: show creators_name: Rebane, AA creators_name: Wang, B creators_name: Ma, L creators_name: Qu, H creators_name: Coleman, J creators_name: Krishnakumar, S creators_name: Rothman, JE creators_name: Zhang, Y title: Two Disease-Causing SNAP-25B Mutations Selectively Impair SNARE C-terminal Assembly ispublished: pub divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F81 keywords: Optical tweezers, SNARE assembly, membrane fusion, protein folding, neuropathy note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. abstract: Synaptic exocytosis relies on assembly of three soluble N-ethylmaleimide-sensitive factor attachment receptor (SNARE) proteins into a parallel four-helix bundle to drive membrane fusion. SNARE assembly occurs by stepwise zippering of the vesicle-associated SNARE (v-SNARE) onto a binary SNARE complex on the target plasma membrane (t-SNARE). Zippering begins with slow N-terminal association followed by rapid C-terminal zippering, which serves as a power stroke to drive membrane fusion. SNARE mutations have been associated with numerous diseases, especially neurological disorders. It remains unclear how these mutations affect SNARE zippering, partly due to difficulties to quantify the energetics and kinetics of SNARE assembly. Here, we used single-molecule optical tweezers to measure the assembly energy and kinetics of SNARE complexes containing single mutations I67T/N in neuronal SNARE synaptosomal-associated protein of 25 kDa (SNAP-25B), which disrupt neurotransmitter release and have been implicated in neurological disorders. We found that both mutations significantly reduced the energy of C-terminal zippering by ~ 10 kBT, but did not affect N-terminal assembly. In addition, we observed that both mutations lead to unfolding of the C-terminal region in the t-SNARE complex. Our findings suggest that both SNAP-25B mutations impair synaptic exocytosis by destabilizing SNARE assembly, rather than stabilizing SNARE assembly as previously proposed. Therefore, our measurements provide insights into the molecular mechanism of the disease caused by SNARE mutations. date: 2018-02-16 date_type: published publisher: ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD official_url: https://doi.org/10.1016/j.jmb.2017.10.012 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green article_type_text: Article verified: verified_manual elements_id: 1504236 doi: 10.1016/j.jmb.2017.10.012 lyricists_name: Krishnakumar, Shyam lyricists_name: Rothman, James lyricists_id: SKRIS94 lyricists_id: JROTH20 actors_name: Krishnakumar, Shyam actors_id: SKRIS94 actors_role: owner full_text_status: public publication: Journal of Molecular Biology volume: 430 number: 4 pagerange: 479-490 pages: 12 issn: 1089-8638 citation: Rebane, AA; Wang, B; Ma, L; Qu, H; Coleman, J; Krishnakumar, S; Rothman, JE; Rebane, AA; Wang, B; Ma, L; Qu, H; Coleman, J; Krishnakumar, S; Rothman, JE; Zhang, Y; - view fewer <#> (2018) Two Disease-Causing SNAP-25B Mutations Selectively Impair SNARE C-terminal Assembly. Journal of Molecular Biology , 430 (4) pp. 479-490. 10.1016/j.jmb.2017.10.012 <https://doi.org/10.1016/j.jmb.2017.10.012>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10049829/1/Rebane%20et%20al_JMB%202018.pdf