Quantification of different iron forms in the aceruloplasminemia brain to explore iron-related neurodegeneration.

ABSTRACT:

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 aceruloplasminemia brain were studied. MRI, EPR and SQUID results that had been previousl

SUBMITTER: Vroegindeweij LHP 

PROVIDER: S-EPMC8055714 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature