By Dr Georg Von Jonquieres

Journal articles

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

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

, 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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Conference Papers

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

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

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

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

Conference Abstracts

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

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

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

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

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

Preprints

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

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