Georg von Jonquieres


Affiliation: University of New South Wales
Country: Australia


  1. Fröhlich D, Suchowerska A, Spencer Z, von Jonquieres G, Klugmann C, Bongers A, et al. In vivocharacterization of the aspartyl-tRNA synthetase DARS: Homing in on the leukodystrophy HBSL. Neurobiol Dis. 2017;97:24-35 pubmed publisher
    ..In summary, our data is an important contribution to a better understanding of DARS function and HBSL pathology. ..
  2. von Jonquieres G, Spencer Z, Rowlands B, Klugmann C, Bongers A, Harasta A, et al. Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy. Acta Neuropathol. 2018;135:95-113 pubmed publisher
    ..This finding signifies the first successful post-symptomatic treatment of a white matter disorder using an adeno-associated virus vector tailored towards oligodendroglial-restricted transgene expression. ..