2023
DOI: 10.1002/mrm.29917
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Quantitative diffusion imaging and genotype‐by‐sex interactions in a rat model of Alexander disease

Nicholas A. Stowe,
Ajay P. Singh,
Brian R. Barnett
et al.

Abstract: PurposeThe clinical diagnosis and classification of Alexander disease (AxD) relies in part on qualitative neuroimaging biomarkers; however, these biomarkers fail to distinguish and discriminate different subtypes of AxD, especially in the presence of overlap in clinical symptoms. To address this gap in knowledge, we applied neurite orientation dispersion and density imaging (NODDI) to an innovative CRISPR‐Cas9 rat genetic model of AxD to gain quantitative insights into the neural substrates and brain microstru… Show more

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“…Specifically, GFAP-targeting ASO treatment for R237H rats at weaning eliminated molecular and cellular damage, delaying the emergence of clinical phenotypes [ 56 ]. The treatment of severely afflicted young adult rats resulted in a significant improvement in total neurologic function, partially reversing motor impairment and white matter impairments, in addition to eliminating pathology [ 56 , 59 ].…”
Section: Alexander Diseasementioning
confidence: 99%
“…Specifically, GFAP-targeting ASO treatment for R237H rats at weaning eliminated molecular and cellular damage, delaying the emergence of clinical phenotypes [ 56 ]. The treatment of severely afflicted young adult rats resulted in a significant improvement in total neurologic function, partially reversing motor impairment and white matter impairments, in addition to eliminating pathology [ 56 , 59 ].…”
Section: Alexander Diseasementioning
confidence: 99%