DOI > 10.5291/ILL-DATA.8-03-1044

This proposal is publicly available since 05/30/2026

Title

Simultaneous SANS and DLS: a powerful combination for elucidating the molecular mechanisms of Wilson's disease

Abstract

Wilson's disease is a condition caused by impaired copper metabolism, ultimately leading to severe consequences such as aggregation of hemoglobin (Hb) and the destruction of red blood cells (erythrocytes). In this experiment, the kinetics, molecular mechanisms and structural changes accompanying these pathologicial processes will be investigated. In addition, the effects of two commonly used treatments for Wilson's disease will be studied on a molecular level. Importantly, the combination of real-time SANS and in-situ DLS will allow to obtain additional dynamic information on Hb aggregation and structural evolution of erythrocyte membranes. The synergy between SANS and DLS will thus enable a comprehensive, quantitative characterisation of the pathological mechanisms underlying Wilson's disease, paving the way towards enhanced treatment options.

Experimental Report

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Data Citation

The recommended format for citing this dataset in a research publication is in the following format:

MATSARSKAIA Olga; SANDRONI Martina and SCHWEINS Ralf. (2021). Simultaneous SANS and DLS: a powerful combination for elucidating the molecular mechanisms of Wilson's disease. Institut Laue-Langevin (ILL) doi:10.5291/ILL-DATA.8-03-1044

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Metadata

Experiment Parameters

  • Environment temperature

    5-37 °C
  • Experiment energy

    6
  • Experiment moment

    USANS & up to 1A-1
  • Experiment res energy

    9%

Sample Parameters

  • Formula

    • Human hemoglobin (Sigma Aldrich)
    • human erythrocytes
    • Zinc chloride
    • Penicillamine
    • Glutathione
    • phosphate buffer in D2O