Dr Georg Von Jonquieres

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I am a molecular and cell biologist with broad neurophysiology and genetics background. My research drives innovative gene therapy development for intractable neurological disorders. Following my Ph.D. at the Centre of Advanced European Studies and Research through the University of Bonn, Germany, I joined UNSW Sydney as research fellow I have grown my passion and expertise developing targeted gene therapies for treatment of intractable diseases in the central nervous system. I enjoy continuously growing my broad understanding in the physiological and neurosciences, particularly in sensory motor neurophysiology, genetic and metabolic diseases and am driven to advance the development of the next generation of smart therapeutics to improve prognosis and treatment outcomes for some of the most devastating pathologies.

Phone

+61 2 9385 2376

E-mail

g.jonquieres@unsw.edu.au

Journal articles | 2025

Pearson LJ; Pinyon JL; Cederholm JME; von Jonquieres G; Bartlett F; Vázquez-Campos X; Delerue F; Ittner LM; Housley GD, 2025, 'Developmental differentiation of mouse inner ear neuron subpopulations resolved with a peripherin-promoter reporter within the Grm8 locus', Scientific Reports, 15, http://dx.doi.org/10.1038/s41598-025-94011-3

Journal articles | 2025

Pinyon JL; von Jonquieres G; Mow SL; Abed AA; Lai K; Manoharan M; Crawford EN; Xue SH; Smith‐Moore S; Caproni LJ; Milsom S; Klugmann M; Lovell NH; Housley GD, 2025, 'Vector‐Free Deep Tissue Targeting of DNA/RNA Therapeutics via Single Capacitive Discharge Conductivity‐Clamped Gene Electrotransfer (Adv. Sci. 3/2025)', Advanced Science, 12, http://dx.doi.org/10.1002/advs.202570016

Journal articles | 2025

Pinyon JL; von Jonquieres G; Mow SL; Abed AA; Lai KY; Manoharan M; Crawford EN; Xue SH; Smith-Moore S; Caproni LJ; Milsom S; Klugmann M; Lovell NH; Housley GD, 2025, 'Vector-Free Deep Tissue Targeting of DNA/RNA Therapeutics via Single Capacitive Discharge Conductivity-Clamped Gene Electrotransfer', Advanced Science, 12, http://dx.doi.org/10.1002/advs.202406545

Journal articles | 2024

, 2024, 'Gene Electrotransfer via Conductivity‐Clamped Electric Field Focusing Pivots Sensori‐Motor DNA Therapeutics: “A Spoonful of Sugar Helps the Medicine Go Down” (Adv. Sci. 30/2024)', Advanced Science, 11

Journal articles | 2024

Parmar J; von Jonquieres G; Gorlamandala N; Chung B; Craig AJ; Pinyon JL; Birnbaumer L; Klugmann M; Moorhouse AJ; Power JM; Housley GD, 2024, 'TRPC Channels Activated by G Protein-Coupled Receptors Drive Ca²⁺ Dysregulation Leading to Secondary Brain Injury in the Mouse Model', Translational Stroke Research, 15, pp. 844 - 858, http://dx.doi.org/10.1007/s12975-023-01173-1

Journal articles | 2024

Pinyon JL; von Jonquieres G; Crawford EN; Abed AA; Power JM; Klugmann M; Browne CJ; Housley DM; Wise AK; Fallon JB; Shepherd RK; Lin JY; McMahon C; McAlpine D; Birman CS; Lai W; Enke YL; Carter PM; Patrick JF; Gay RD; Marie C; Scherman D; Lovell NH; Housley GD, 2024, 'Gene Electrotransfer via Conductivity-Clamped Electric Field Focusing Pivots Sensori-Motor DNA Therapeutics: “A Spoonful of Sugar Helps the Medicine Go Down”', Advanced Science, 11, http://dx.doi.org/10.1002/advs.202401392

Journal articles | 2022

Cederholm JME; Parley KE; Perera CJ; von Jonquieres G; Pinyon JL; Julien JP; Ryugo DK; Ryan AF; Housley GD, 2022, 'Noise-induced hearing loss vulnerability in type III intermediate filament peripherin gene knockout mice', Frontiers in Neurology, 13, http://dx.doi.org/10.3389/fneur.2022.962227

Journal articles | 2022

Fröhlich D; Kalotay E; von Jonquieres G; Bongers A; Lee B; Suchowerska AK; Housley GD; Klugmann M, 2022, 'Dual-function AAV gene therapy reverses late-stage Canavan disease pathology in mice', Frontiers in Molecular Neuroscience, 15, http://dx.doi.org/10.3389/fnmol.2022.1061257

Journal articles | 2021

Eykens C; Rossaert E; Duqué S; Rué L; Bento-Abreu A; Hersmus N; Lenaerts A; Kerstens A; Corthout N; Munck S; Van Damme P; Holt MG; von Jonquires G; Klugmann M; Van Den Bosch L; Robberecht W, 2021, 'AAV9-mediated gene delivery of MCT1 to oligodendrocytes does not provide a therapeutic benefit in a mouse model of ALS', Molecular Therapy Methods and Clinical Development, 20, pp. 508 - 519, http://dx.doi.org/10.1016/j.omtm.2021.01.006

Journal articles | 2021

von Jonquieres G; Rae CD; Housley GD, 2021, 'Emerging Concepts in Vector Development for Glial Gene Therapy: Implications for Leukodystrophies', Frontiers in Cellular Neuroscience, 15, pp. 661857, http://dx.doi.org/10.3389/fncel.2021.661857

Journal articles | 2020

Brown CN; Fultz EK; Ferdousian S; Rogers S; Lustig E; Page A; Shahin JR; Flaherty DM; Von Jonquieres G; Bryant CD; Kippin TE; Szumlinski KK, 2020, 'Transgenic Analyses of Homer2 Function Within Nucleus Accumbens Subregions in the Regulation of Methamphetamine Reward and Reinforcement in Mice', Frontiers in Psychiatry, 11, http://dx.doi.org/10.3389/fpsyt.2020.00011

Journal articles | 2020

Housley DM; Pinyon JL; Von Jonquieres G; Perera CJ; Smout M; Liddell MJ; Jennings EA; Wilson D; Housley GD, 2020, 'Australian scorpion Hormurus waigiensis venom fractions show broad bioactivity through modulation of bio-impedance and cytosolic calcium', Biomolecules, 10, http://dx.doi.org/10.3390/biom10040617

Journal articles | 2020

Housley GD; von Jonquieres G; Pinyon JL; Matheson JAT; Pearson LJ; Salthouse TP; Cederholm JM, 2020, 'Cochlear homeostasis: a molecular physiological perspective on maintenance of sound transduction and auditory neurotransmission with noise and ageing', Current Opinion in Physiology, 18, pp. 106 - 115, http://dx.doi.org/10.1016/j.cophys.2020.09.012

Journal articles | 2020

Richards LA; Kumari A; Knezevic K; Thoms JAI; von Jonquieres G; Napier CE; Ali Z; O'Brien R; Marks-Bluth J; Maritz MF; Pickett HA; Morris J; Pimanda JE; MacKenzie KL, 2020, 'DKC1 is a transcriptional target of GATA1 and drives upregulation of telomerase activity in normal human erythroblasts', Haematologica, 105, pp. 1517 - 1526, http://dx.doi.org/10.3324/haematol.2018.215699

Journal articles | 2019

Campbell RR; Domingo RD; Williams AR; Wroten MG; McGregor HA; Waltermire RS; Greentree DI; Goulding SP; Thompson AB; Lee KM; Quadir SG; Chavez CLJ; Coelho MA; Gould AT; Von Jonquieres G; Klugmann M; Worley PF; Kippin TE; Szumlinski KK, 2019, 'Increased alcohol-drinking induced by manipulations of mGlu5 phosphorylation within the bed nucleus of the stria terminalis', Journal of Neuroscience, 39, pp. 2745 - 2761, http://dx.doi.org/10.1523/JNEUROSCI.1909-18.2018

Journal articles | 2019

Cederholm JME; Kim Y; von Jonquieres G; Housley GD, 2019, 'Human Brain Region-Specific Alternative Splicing of TRPC3, the Type 3 Canonical Transient Receptor Potential Non-Selective Cation Channel', Cerebellum, 18, pp. 536 - 543, http://dx.doi.org/10.1007/s12311-019-01026-4

Journal articles | 2019

Pinyon JL; von Jonquieres G; Crawford EN; Duxbury M; Al Abed A; Lovell NH; Klugmann M; Wise AK; Fallon JB; Shepherd RK; Birman CS; Lai W; McAlpine D; McMahon C; Carter PM; Enke YL; Patrick JF; Schilder AGM; Marie C; Scherman D; Housley GD, 2019, 'Neurotrophin gene augmentation by electrotransfer to improve cochlear implant hearing outcomes', Hearing Research, 380, pp. 137 - 149, http://dx.doi.org/10.1016/j.heares.2019.06.002

Journal articles | 2018

Broccoli L; Uhrig S; von Jonquieres G; Schönig K; Bartsch D; Justice NJ; Spanagel R; Sommer WH; Klugmann M; Hansson AC, 2018, 'Targeted overexpression of CRH receptor subtype 1 in central amygdala neurons: effect on alcohol-seeking behavior', Psychopharmacology, 235, pp. 1821 - 1833, http://dx.doi.org/10.1007/s00213-018-4908-6

Journal articles | 2018

Fröhlich D; Suchowerska AK; Voss C; He R; Wolvetang E; Von Jonquieres G; Simons C; Fath T; Housley GD; Klugmann M; Froehlich D, 2018, 'Expression pattern of the aspartyl-tRNA synthetase DARS in the human brain', Frontiers in Molecular Neuroscience, 11, pp. 81, http://dx.doi.org/10.3389/fnmol.2018.00081

Journal articles | 2018

Powell KL; Fitzgerald X; Shallue C; Jovanovska V; Klugmann M; Von Jonquieres G; O'Brien TJ; Morris MJ, 2018, 'Gene therapy mediated seizure suppression in Genetic Generalised Epilepsy: Neuropeptide Y overexpression in a rat model', Neurobiology of Disease, 113, pp. 23 - 32, http://dx.doi.org/10.1016/j.nbd.2018.01.016

Journal articles | 2018

von Jonquieres G; Spencer ZHT; Rowlands BD; Klugmann CB; Bongers A; Harasta AE; Parley KE; Cederholm J; Teahan O; Pickford R; Delerue F; Ittner LM; Fröhlich D; McLean CA; Don AS; Schneider M; Housley GD; Rae CD; Klugmann M; Froehlich D, 2018, 'Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy', Acta Neuropathologica, 135, pp. 95 - 113, http://dx.doi.org/10.1007/s00401-017-1784-9

Journal articles | 2017

Bi M; Gladbach A; Van Eersel J; Ittner A; Przybyla M; Van Hummel A; Chua SW; Hoven JVD; Lee WS; Müller J; Parmar J; Jonquieres GV; Stefen H; Guccione E; Fath T; Housley GD; Klugmann M; Ke YD; Ittner LM, 2017, 'Tau exacerbates excitotoxic brain damage in an animal model of stroke', Nature Communications, 8, http://dx.doi.org/10.1038/s41467-017-00618-0

Journal articles | 2017

Datko MC; Hu JH; Williams M; Reyes CM; Lominac KD; von Jonquieres G; Klugmann M; Worley PF; Szumlinski KK, 2017, 'Behavioral and neurochemical phenotyping of mice incapable of homer1a induction', Frontiers in Behavioral Neuroscience, 11, pp. 208, http://dx.doi.org/10.3389/fnbeh.2017.00208

Journal articles | 2017

Fröhlich D; Suchowerska AK; Spencer ZHT; von Jonquieres G; Klugmann CB; Bongers A; Delerue F; Stefen H; Ittner LM; Fath T; Housley GD; Klugmann M, 2017, 'In vivo characterization of the aspartyl-tRNA synthetase DARS: Homing in on the leukodystrophy HBSL', Neurobiology of Disease, 97, pp. 24 - 35, http://dx.doi.org/10.1016/j.nbd.2016.10.008

Journal articles | 2017

Georgiou E; Sidiropoulou K; Richter J; Papaneophytou C; Sargiannidou I; Kagiava A; Von Jonquieres G; Christodoulou C; Klugmann M; Kleopa KA, 2017, 'Gene therapy targeting oligodendrocytes provides therapeutic benefit in a leukodystrophy model', Brain, 140, pp. 599 - 616, http://dx.doi.org/10.1093/brain/aww351

Journal articles | 2017

Szumlinski KK; Lominac KD; Campbell RR; Cohen M; Fultz EK; Brown CN; Miller BW; Quadir SG; Martin D; Thompson AB; von Jonquieres G; Klugmann M; Phillips TJ; Kippin TE, 2017, 'Methamphetamine Addiction Vulnerability: The Glutamate, the Bad, and the Ugly', Biological Psychiatry, 81, pp. 959 - 970, http://dx.doi.org/10.1016/j.biopsych.2016.10.005

Journal articles | 2016

O'Brien R; Tran SL; Maritz MF; Liu B; Kong CF; Purgato S; Yang C; Murray J; Russell AJ; Flemming CL; von Jonquieres G; Pickett HA; London WB; Haber M; Gunaratne PH; Norris MD; Perini G; Fletcher JI; MacKenzie KL, 2016, 'MYC-Driven Neuroblastomas Are Addicted to a Telomerase-Independent Function of Dyskerin', Cancer Research, http://dx.doi.org/10.1158/0008-5472.CAN-15-0879

Journal articles | 2016

von Jonquieres G; Fröhlich D; Klugmann CB; Wen X; Harasta AE; Ramkumar R; Spencer ZHT; Housley GD; Klugmann M, 2016, 'Recombinant human myelin-associated glycoprotein promoter drives selective AAV-mediated transgene expression in oligodendrocytes', Frontiers in Molecular Neuroscience, 9, http://dx.doi.org/10.3389/fnmol.2016.00013

Journal articles | 2015

Gould AT; Sacramento AD; Wroten MG; Miller BW; Von Jonquieres G; Klugmann M; Ben-Shahar O; Szumlinski KK, 2015, 'Cocaine-elicited imbalances in ventromedial prefrontal cortex Homer1 versus Homer2 expression: Implications for relapse', Addiction Biology, 20, pp. 148 - 157, http://dx.doi.org/10.1111/adb.12088

Journal articles | 2015

Harasta AE; Power JM; Von Jonquieres G; Karl T; Drucker DJ; Housley GD; Schneider M; Klugmann M, 2015, 'Septal glucagon-like peptide 1 receptor expression determines suppression of cocaine-induced behavior', Neuropsychopharmacology, 40, pp. 1969 - 1978, http://dx.doi.org/10.1038/npp.2015.47

Journal articles | 2014

Cozzoli DK; Courson J; Wroten MG; Greentree DI; Lum EN; Campbell RR; Thompson AB; Maliniak D; Worley PF; Jonquieres G; Klugmann M; Finn DA; Szumlinski KK, 2014, 'Binge alcohol drinking by mice requires intact group1 metabotropic glutamate receptor signaling within the Central nucleus of the Amygdale', Neuropsychopharmacology, 39, pp. 435 - 444, http://dx.doi.org/10.1038/npp.2013.214

Journal articles | 2014

Von Jonquieres G; Froud KE; Klugmann CB; Wong ACY; Housley GD; Klugmann M, 2014, 'Loss of central auditory processing in a mouse model of canavan disease', PLoS ONE, 9, http://dx.doi.org/10.1371/journal.pone.0097374

Journal articles | 2013

Ary AW; Lominac KD; Wroten MG; Williams AR; Campbell R; Ben-shahar OM; Von Jonquieres G; Klugmann M; Szumlinski KK, 2013, 'Imbalances in prefrontal cortex CC-Homer1 versus CC-Homer2 expression promote cocaine preference', Journal of Neuroscience, 33, pp. 8101 - 8113, http://dx.doi.org/10.1523/JNEUROSCI.1727-12.2013

Journal articles | 2013

Jonquieres G; Mersmann N; Teahan O; Klugmann CB; Harasta AE; Lutz B; Housley GD; Klugmann M, 2013, 'AAV-BASED GENE THERAPY APPROACHES FOR THE TREATMENT OF CANAVAN DISEASE', CYTOTHERAPY, 15, pp. S12 - S12, http://dx.doi.org/10.1016/j.jcyt.2013.01.042

Journal articles | 2013

Obara I; Goulding SP; Gould AT; Lominac KD; Hu JH; Zhang PW; von Jonquieres G; Dehoff M; Xiao B; Seeburg PH; Worley PF; Klugmann M; Szumlinski KK, 2013, 'Homers at the interface between reward and pain', Frontiers in Psychiatry, 4, http://dx.doi.org/10.3389/fpsyt.2013.00039

Journal articles | 2013

Von Jonquieres G; Mersmann N; Klugmann CB; Harasta AE; Lutz B; Teahan O; Housley GD; Frohlich D; Kramer-Albers EM; Klugmann M, 2013, 'Glial Promoter Selectivity following AAV-Delivery to the Immature Brain', PLoS ONE, 8, pp. Article number e65646, http://dx.doi.org/10.1371/journal.pone.0065646

Journal articles | 2013

von Jonquieres G; Klugmann C; Harrasta A; Klugmann M, 2013, 'DEVELOPMENT OF RAAV VECTORS FOR THE TREATMENT OF LEUKODYSTROPHIES', CYTOTHERAPY, 15, pp. S33 - S33, http://dx.doi.org/10.1016/j.jcyt.2013.01.125

Journal articles | 2011

Thoms JAI; Birger Y; Foster S; Knezevic K; Kirshenbaum Y; Chandrakanthan V; Jonquieres G; Spensberger D; Wong JW; Oram SH; Kinston SJ; Groner Y; Lock RB; MacKenzie KL; Gottgens B; Izraeli S; Pimanda JE, 2011, 'ERG promotes T-acute lymphoblastic leukemia and is transcriptionally regulated in leukemic cells by a stem cell enhancer', Blood, 117, pp. 7079 - 7089, http://dx.doi.org/10.1182/blood-2010-12-317990

Journal articles | 2010

Marshall GM; Gherardi S; Xu N; Neiron Z; Trahair TN; Scarlett CJ; Chang DK; Liu PY; Jankowski K; Iraci N; Haber M; Norris MD; Keating J; Sekyere E; Jonquieres G; Biankin AV; Stossi F; Katzenellenbogen BS; Perini G; Liu T, 2010, 'Transcriptional upregulation of histone deacetylase 2 promotes Myc-induced oncogenic effects', Oncogene, 29, pp. 5957 - 5968, http://dx.doi.org/10.1038/onc.2010.332

Journal articles | 2010

Von Jonquieres G, 2010, 'Transcriptional upregulation of histone deacetylase 2 promotes Myc-induced oncogenic effects', Oncogene, 29, pp. 5957 - 5968, http://dx.doi.org/10.1038/onc.2010.332

Journal articles | 2009

Von Jonquieres G, 2009, 'Hyperactivation of the Insulin-like Growth Factor Receptor I Signaling Pathway Is an Essential Event for Cisplatin Resistance of Ovarian Cancer Cells', Cancer Research, 69, pp. 2996 - 3003, http://dx.doi.org/10.1158/0008-5472.CAN-08-3153

Journal articles | 2008

Von Jonquieres G, 2008, 'Acquired cisplatin resistance in the head–neck cancer cell line Cal27 is associated with decreased DKK1 expression and can partially be reversed by overexpression of DKK1', International Journal of Cancer, 123, pp. 2013 - 2019, http://dx.doi.org/10.1002/ijc.23721

Journal articles | 2008

Von Jonquieres G, 2008, 'Epidermal growth factor receptor pathway analysis identifies amphiregulin as a key factor for cisplatin resistance of human breast cancer cells.', The Journal of Biological Chemistry, 283, pp. 739 - 750, http://dx.doi.org/10.1074/jbc.M706287200
Preprints | 2021

Cederholm JME; Parley KE; Perera CJ; von Jonquieres G; Pinyon JL; Julien J-P; Ryugo DK; Ryan AF; Housley GD, 2021, Association of cochlear outer hair cell - type II spiral ganglion afferents with protection from noise-induced hearing loss, http://dx.doi.org/10.1101/2021.11.06.467554

Conference Abstracts | 2020

Pearson L; Pinyon J; Von Jonquieres G; Cederholm J; Housley G, 2020, 'Characterising auditory neurons through transgene expression.', in Australasian Auditory Neuroscience Webinar, presented at Australasian Auditory Neuroscience Workshop

Conference Abstracts | 2017

von Jonquieres G; Klugmann C; Frohlich D; Housley GD; Klugmann M, 2017, 'NOVEL APPROACHES TO PREVENT THE NAASTY - TARGETED GENE THERAPY FOR LEUKODYSTROPHIES', in JOURNAL OF GENE MEDICINE, WILEY, AUSTRALIA, Univ Technol Sydney, Sydney, Vol. 20, presented at Joint 10th Annual Scientific Meeting of the Australian-Gene-and-Cell-Therapy-Society (AGCTS) and Australasian-Society-for-Stem-Cell-Research (ASSCR), AUSTRALIA, Univ Technol Sydney, Sydney, 24 May 2017 - 26 May 2017, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000426528300030&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Papers | 2016

Georgiou E; Kagiava A; Sidiropoulou K; Richter J; Sargiannidou I; Papaneophytou C; von Jonquieres G; Christodoulou C; Klugmann M; Kleopa K, 2016, 'Gene delivery to oligodendrocytes re-establishes gap junctions and rescues a hypomyelinating leukodystrophy model', in EUROPEAN JOURNAL OF NEUROLOGY, WILEY, pp. 694 - 695, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000431634203169&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Papers | 2016

Perera C; Von Jonquieres G; Pinyon J; Parley K; Cederholm J; Julien J-P; Ryan A; Housley G, 2016, 'Phenotyping the differential innervation of the peripherin knockout mouse cochlea', in Proceedings of the Australian Physiological Society, Adelaide, presented at Australian Physiological Society, Adelaide, 04 December 2016 - 07 December 2016

Conference Abstracts | 2015

Harasta A; Power JM; von Jonquieres G; Karl T; Drucker DJ; Housley GD; Schneider M; Klugmann M, 2015, 'Septal glucagon-like peptide 1 receptor expression determines suppression of cocaine-induced behaviour', in JOURNAL OF NEUROCHEMISTRY, WILEY-BLACKWELL, AUSTRALIA, Cairns, Vol. 134, pp. 214 - 214, presented at 25th Biennial Meeting of the International-Society-for-Neurochemistry Jointly with the 13th Meeting of the Asian-Pacific-Society-for-Neurochemistry in Conjunction with the 35th Meeting of the Australasian-Neuroscience-Society, AUSTRALIA, Cairns, 23 August 2015 - 27 August 2015, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000360206300522&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Abstracts | 2015

Harrison-Tikisci Z; von Jonquieres G; Rowlands B; Klugmann C; Delarue F; Ittner L; Pickford R; Housley G; Rae C; Klugmann M, 2015, 'N-acetylaspartate: genomics, phenomics and metabonomics', in JOURNAL OF NEUROCHEMISTRY, WILEY-BLACKWELL, AUSTRALIA, Cairns, Vol. 134, pp. 141 - 141, presented at 25th Biennial Meeting of the International-Society-for-Neurochemistry Jointly with the 13th Meeting of the Asian-Pacific-Society-for-Neurochemistry in Conjunction with the 35th Meeting of the Australasian-Neuroscience-Society, AUSTRALIA, Cairns, 23 August 2015 - 27 August 2015, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000360206300323&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Abstracts | 2015

Jonquieres GV; Mersmann N; Klugmann C; Harrasta A; Klugmann M, 2015, 'Targeting oligodendrocytes in rAAV mediated gene replacement therapy for Canavan disease', in JOURNAL OF NEUROCHEMISTRY, WILEY-BLACKWELL, AUSTRALIA, Cairns, Vol. 134, pp. 289 - 289, presented at 25th Biennial Meeting of the International-Society-for-Neurochemistry Jointly with the 13th Meeting of the Asian-Pacific-Society-for-Neurochemistry in Conjunction with the 35th Meeting of the Australasian-Neuroscience-Society, AUSTRALIA, Cairns, 23 August 2015 - 27 August 2015, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000360206300729&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Papers | 2013

von Jonquieres G; Klugmann C; Harrasta A; Klugmann M, 2013, 'DEVELOPMENT OF rAAV VECTORS FOR THE TREATMENT OF LEUKODYSTROPHIES', in JOURNAL OF GENE MEDICINE, WILEY-BLACKWELL, AUSTRALIA, Univ Technol, Sydney, pp. 334 - 335, presented at 8th Meeting of the Australasian-Gene-Therapy-Society, AUSTRALIA, Univ Technol, Sydney, 08 May 2013 - 10 May 2013, https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000325102500046&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=891bb5ab6ba270e68a29b250adbe88d1

Conference Papers | 2012

Kumari A; Richards LA; Jonquieres G; Knezevic K; O'Brien R; Napier CE; Marks-Bluth J; Pickett H; Pimanda JE; MacKenzie KL, 2012, 'Dyskerin Is Upregulated During Erythroid Differentiation of Human Hematopoietic Progenitor Cells and Hyperactivates Telomerase in Erythroid Precursor Cells', in BLOOD, AMER SOC HEMATOLOGY, GA, Atlanta, presented at 54th Annual Meeting and Exposition of the American-Society-of-Hematology (ASH), GA, Atlanta, 08 December 2012 - 11 December 2012, http://dx.doi.org/10.1182/blood.V120.21.980.980

Establishing Vision-BaDGE anti-VEGF DNA therapy for age-related macular degeneration. Australia's Economic Accelerator Seed Grants (2023 - 2024)

Bionic-array Directed Gene Electrotransfer for Treating Focal Brain Disorders. NHMRC Ideas Grant (2021 - 2025)

Imaging and analysis of drug mode of action in pre-clinical traumatic Brain Injury Models. Research Contracts (2024)

Restoring hearing by gene electrotransfer - based enablement of auditory neurons to transduce sound. NHMRC Ideas Grant (2020 - 2022)

Development of next generation DNA therapeutics for the treatment of Canavan Disease. European Leukodystrophy Association Research Grant (2016)

Following my Ph.D. research in Germany, I joined the Translational Neuroscience Facility at UNSW Sydney with a keen interest to develop safe and efficient therapeutic interventions for a variety of devastating intractable neurological indications based on a smart intervention in underlying inter- and intracellular signaling cascades. To this end, we develop viral and non-viral gene therapies to achieve spatially and temporally controlled expression of therapeutic payloads directed precisely to the required cell type and sub-cellular compartment. Within our non-viral drug/device development scheme we collaborate closely with exerts in the Graduate School of Biomedical Engineering (GSBME) continuing to refine and reposition our cutting-edge Bionic array directed gene electrotransfer (BaDGE®) platform technology.

The main pillars of our BaDGE® program include:

Brain-BaDGE: Achieved a world first focal deep tissue in vivo vector free gene transfer to neurons with a needle-like device. This project seeks to translate focal electro-lens field focusing technology using miniscule charge into clinical practice for treatment of drug-resistant focal epilepsy and other neurological indications that require precise DNA/RNA targeting in the CNS. This program is an international research collaboration with leading clinical researchers from the Experimental Epileptology group at the Hertie Institute for clinical brain research at the University of Tubingen, Germany and supported by our NHMRC grant and our industry partner Boehringer Ingelheim Research Beyond Boarders GmbH.

Vision-BaDGE: Develops gene electrotransfer therapy to improve current treatment practice for wet age - related macular degeneration and diabetic retinopathy by replacing the frequent intravitreal injections with a safer, efficient, and much longer lasting approach. This program and leverages our industry connections with Boehringer Ingelheim Research Beyond Boarders GmbH and Touchlight Genetics Inc and is supported by an Australian Economic Accelerator grant.

Hearing-BaDGE: This flagship technology modified the Cochlear^TM Hearing Implant to enable focal neurotrophin gene delivery to guide neurite growth towards the bionic implant electrodes, closing the 'neural gap' and improving cochlear implant performance. This technology is currently progressing through PhaseI/IIa first-in-human clinical trial.

Muscle-BaDGE: This program positions our needle-like gene electrotransfer device with electro-lens field focusing technology into a disposable single use device for modern mRNA and DNA immunotherapy and vaccine delivery.

Nerve-BaDGE: Aims to promote nerve regeneration and repair following trauma such as spinal chord injury.

Our Brain Injury Small Molecule Therapeutics Program program is an integral part of a vibrant three way preclinical research collaboration between our UNSW Sydney team, the local neuroscience and drug development company Nyrada Inc, and a Brain Trauma Neuroprotection team based in the USA. Together we drive the development of novel treatments that aim to limit secondary brain injury expansion within the 'golden therapeutic window' following a stroke or traumatic injury.

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