SKM 2021 – wissenschaftliches Programm

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BP: Fachverband Biologische Physik

BP 3: Protein Structure and Dynamics

BP 3.3: Vortrag

Donnerstag, 30. September 2021, 10:45–11:00, H1

Scattering techniques: powerful tools to elucidate the molecular mechanisms of Wilson's disease — •Olga Matsarskaia — Institut Laue-Langevin, Grenoble, France

Copper (Cu) is an essential element for mammals and its metabolism is thus tightly regulated [1]. In the case of Wilson's disease, however, Cu metabolism is impaired, leading to abnormal Cu levels in the body [2]. Severe, often lethal, consequences ensue, such as liver and neurological damage [3,4] as well as the destruction of hemoglobin (Hb) and red blood cells (RBCs) [5]. The latter two symptoms are believed to be due to Cu-induced aggregation of Hb [6,7]. The current understanding of Wilson's disease is predominantly phenomenological. Thus, this project applies an interdisciplinary array of techniques including neutron and X-ray scattering to deepen the understanding of this highly complex condition. Scattering data recently obtained using human RBCs and purified human Hb will be presented, demonstrating real-time effects of Cu addition to these systems. The results obtained will be discussed in the broader context of medical research with the goal of inspiring an interdisciplinary dialogue between fundamental science and clinical applications.

[1] Löffler & Petrides, Springer Heidelberg (2007); [2] Riordan & Roger, J.Hepatol. (2001) 34, 433-48; [3] Ala et al., The Lancet (2007), 369, 397-408; [4] Gitlin, Gastroenterol. (2003), 125, 1868-77; [5] Ferenci, Metab. Brain Dis. (2004), 19, 229-39; [6] Rifkind, Blood (1965), 26, 433-48; [7] Jandl, Engle, Allen, J. Clin. Invest. (1960), 39, 1818-36