Isoprostanoid Plasma Levels Are Relevant to Cerebral Adrenoleukodystrophy Disease.

ABSTRACT: Cerebral adrenoleukodystrophy (ALD) is a rare neuroinflammatory disorder characterized by progressive demyelination. Mutations within the ABCD1 gene result in very long-chain fatty acid (VLCFA) accumulation within the peroxisome, particularly in the brain. While this VLCFA accumulation is known to be the driving cause of the disease, oxidative stress can be a contributing factor. For patients with early cerebral disease, allogeneic hematopoietic stem cell transplantation (HSCT) is the standard of care, and this can be supported by antioxidants. To evaluate the involvement of fatty acid oxidation in the disease, F₂-isoprostanes (F₂-IsoPs), F_2-dihomo-isoprostanes (F₂-dihomo-IsoPs) and F₄-neuroprostanes (F₄-NeuroPs)-whic

SUBMITTER: Signorini C 

PROVIDER: S-EPMC8874514 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature