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Dr Gabriele Kaminski Schierle

University Position
Senior Research Associate

Interests

Our current research is focused on uncovering the molecular mechanisms that cause proteins to misfold and aggregate in live model systems of Alzheimer?s Disease (AD) and Parkinson?s disease (PD). To enable this work we have developed a range of advanced optical imaging techniques that permit us to look at these processes with unprecedented detail. Two recent developments are key for this research: 1) Amyloids develop an intrinsic fluorescence that reports on the aggregation state of neurotoxic proteins This discovery has permitted us to design highly specific and quantitative in vivo sensors of amyloidogenesis (see Kaminski Schierle et al., 2011) 2) direct Stochastic Optical Reconstruction Microscopy (dSTORM) quantifies the morphology of toxic species in cell models of disease We were the first group able to visualise the morphology of aggregate species and their processing in situ in neuronal cells, at a resolution down to 10 nm (see Kaminski Schierle et al., 2011)

Key Publications

Mutation of the ALS/FTD-associated RNA-binding protein FUS affects axonal development.

DOI: http://doi.org/10.1523/JNEUROSCI.2148-23.2024
Journal: J Neurosci
E-pub date: 3 Jul 2024
Authors: FWV Tartwijk, LCS Wunderlich, I Mela, S Makarchuk, MAH Jakobs, S Qamar, K Franze, GSK Schierle, PHS George-Hyslop, JQ Lin, CE Holt, CF Kaminski

The structure and global distribution of the endoplasmic reticulum network are actively regulated by lysosomes.

DOI: http://doi.org/10.1126/sciadv.abc7209
Journal: Sci Adv
E-pub date: 1 Dec 2020
Authors: M Lu, FW van Tartwijk, JQ Lin, W Nijenhuis, P Parutto, M Fantham, CN Christensen, E Avezov, CE Holt, A Tunnacliffe, D Holcman, L Kapitein, GSK Schierle, CF Kaminski

Phase Separation of FUS is Modulated by Methylation State of Cation-π Interactions and Interaction with TNPO1

DOI: http://doi.org/10.1016/j.cell.2018.03.056
Journal: Cell
E-pub date: 19 Apr 2018
Authors: PH St George-Hyslop, S Qamar, G Wang, SJ Randle, FS Ruggeri, J Varela, CF Kaminski, GS Kaminski, M Vendruscolo, TPJ Knowles, D Klenerman, CE Holt, Q Lin, W meadows

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function.

DOI: http://doi.org/10.1016/j.neuron.2015.10.030
Journal: Neuron
E-pub date: 18 Nov 2015
Authors: T Murakami, S Qamar, JQ Lin, GSK Schierle, E Rees, A Miyashita, AR Costa, RB Dodd, FTS Chan, CH Michel, D Kronenberg-Versteeg, Y Li, S-P Yang, Y Wakutani, W Meadows, RR Ferry, L Dong, GG Tartaglia, G Favrin, W-L Lin, DW Dickson, M Zhen, D Ron, G Schmitt-Ulms, PE Fraser, NA Shneider, C Holt, M Vendruscolo, CF Kaminski, P St George-Hyslop

Publications

ERnet: a tool for the semantic segmentation and quantitative analysis of endoplasmic reticulum topology.

DOI: http://doi.org/10.1038/s41592-023-01815-0
Journal: Nat Methods
E-pub date: 1 Apr 2023
Authors: M Lu, CN Christensen, JM Weber, T Konno, NF Läubli, KM Scherer, E Avezov, P Lio, AA Lapkin, GS Kaminski Schierle, CF Kaminski

Intracellular Aβ42 aggregation leads to cellular thermogenesis

DOI: http://doi.org/10.1101/2022.03.30.486355
Journal:
E-pub date: 1 Aug 2022
Authors: CW Chung, A Stephens, T Konno, E Ward, E Avezov, C Kaminski, A Hassanali, G Kaminski Schierle

Intracellular Aβ42 Aggregation Leads to Cellular Thermogenesis.

DOI: http://doi.org/10.1021/jacs.2c03599
Journal: J Am Chem Soc
E-pub date: 8 Jun 2022
Authors: CW Chung, AD Stephens, T Konno, E Ward, E Avezov, CF Kaminski, AA Hassanali, GS Kaminski Schierle

A waveguide imaging platform for live-cell TIRF imaging of neurons over large fields of view.

DOI: http://doi.org/10.1002/jbio.201960222
Journal: J Biophotonics
E-pub date: 1 Jun 2020
Authors: IS Opstad, F Ströhl, M Fantham, C Hockings, O Vanderpoorten, FW van Tartwijk, JQ Lin, J-C Tinguely, FT Dullo, GS Kaminski-Schierle, BS Ahluwalia, CF Kaminski

α-Synuclein – Regulator of Exocytosis, Endocytosis, or Both?

DOI: http://doi.org/10.1016/j.tcb.2017.02.002
Journal: Trends Cell Biol
E-pub date: 1 Jul 2017
Authors: J Lautenschläger, CF Kaminski, GS Kaminski Schierle

Lifetime imaging of a fluorescent protein sensor reveals surprising stability of ER thiol redox.

DOI: http://doi.org/10.1083/jcb.201211155
Journal: J Cell Biol
E-pub date: 15 Apr 2013
Authors: E Avezov, BCS Cross, GS Kaminski Schierle, M Winters, HP Harding, EP Melo, CF Kaminski, D Ron

Protein amyloids develop an intrinsic fluorescence signature during aggregation.

DOI: http://doi.org/10.1039/c3an36798c
Journal: Analyst
E-pub date: 7 Apr 2013
Authors: FTS Chan, GS Kaminski Schierle, JR Kumita, CW Bertoncini, CM Dobson, CF Kaminski

Analysis of the native structure, stability and aggregation of biotinylated human lysozyme.

DOI: http://doi.org/10.1371/journal.pone.0050192
Journal: PLoS One
E-pub date: 1 Aug 2012
Authors: M Ahn, E De Genst, GS Kaminski Schierle, M Erdelyi, CF Kaminski, CM Dobson, JR Kumita

Towards multiparametric fluorescent imaging of amyloid formation: studies of a YFP model of alpha-synuclein aggregation.

DOI: http://doi.org/10.1016/j.jmb.2009.10.066
Journal: J Mol Biol
E-pub date: 22 Jan 2010
Authors: TJ van Ham, A Esposito, JR Kumita, S-TD Hsu, GS Kaminski Schierle, CF Kaminski, CM Dobson, EAA Nollen, CW Bertoncini