Quantification of different iron forms in the aceruloplasminemia brain to explore iron-related neurodegeneration.
Lena HP Vroegindeweij, Lucia Bossoni, Agnita JW Boon, JH Paul Wilson, Marjolein Bulk, Jacqueline Labra-Muñoz, Martina Huber, Andrew Webb, Louise van der Weerd, Janneke G Langendonk
Neuroimage Clin | Published : 2021
AIMS: Aceruloplasminemia is an ultra-rare neurodegenerative disorder associated with massive brain iron deposits, of which the molecular composition is unknown. We aimed to quantitatively determine the molecular iron forms in the aceruloplasminemia brain, and to illustrate their influence on iron-sensitive MRI metrics. METHODS: The inhomogeneous transverse relaxation rate (R2*) and magnetic susceptibility obtained from 7 T MRI were combined with Electron Paramagnetic Resonance (EPR) and Superconducting Quantum Interference Device (SQUID) magnetometry. The basal ganglia, thalamus, red nucleus, dentate nucleus, superior- and middle temporal gyrus and white matter of a post-mortem aceruloplasmi..View full abstract