2020
DOI: 10.1107/s1600577520006785
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X-ray phase contrast tomography for the investigation of amyotrophic lateral sclerosis

Abstract: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting motor neurons. Pre-clinical studies drive the development of animal models that well mimic ALS disorder and enable both the dissection of disease processes and an early assessment of therapy efficacy. A comprehensive knowledge of neuronal and vascular lesions in the brain and spinal cord is an essential factor to understand the development of the disease. Spatial resolution and bidimensional imaging are important … Show more

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Cited by 10 publications
(8 citation statements)
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“…A key ability of XPCT is the possibility to generate a 3D multiscale image of the whole brain, which displays information on the NN and VN simultaneously ( Fratini et al, 2015 ; Bukreeva et al, 2017 ; Begani Provinciali et al, 2020 ). In previous works ( Cedola et al, 2017 ; Massimi et al, 2019 , 2020 ), we exploited this unique feature of XPCT to evaluate morphological alterations in the VN and NN, both in EAE and in the APP/PS1dE9 mouse model of Alzheimer’s disease (AD).…”
Section: Introductionmentioning
confidence: 99%
“…A key ability of XPCT is the possibility to generate a 3D multiscale image of the whole brain, which displays information on the NN and VN simultaneously ( Fratini et al, 2015 ; Bukreeva et al, 2017 ; Begani Provinciali et al, 2020 ). In previous works ( Cedola et al, 2017 ; Massimi et al, 2019 , 2020 ), we exploited this unique feature of XPCT to evaluate morphological alterations in the VN and NN, both in EAE and in the APP/PS1dE9 mouse model of Alzheimer’s disease (AD).…”
Section: Introductionmentioning
confidence: 99%
“…Indeed, the dependency on fat oxidation is increased in ALS muscle cells, compared to healthy ones, suggesting a cell autonomous defect in energy metabolism [ 77 ]. In line with this observation, muscle-specific expression of mutated SOD1 G93A causes mitochondria abnormalities and dysfunction in vivo [ 82 , 83 ], parallel to neurovascular alterations, observed through X-ray-Phase-Contrast-Tomography (XPCT), at different stages of the disease [ 84 ]. XPCT demonstrated the ability to detect neuron alterations even in a pre-symptomatic state in SOD1 G93A mice.…”
Section: Metabolic Alteration In Neurodegenerative Diseasesmentioning
confidence: 93%
“…ALS is the most common and severe form of motor neuron diseases involving localized muscle weakness and respiratory symptoms. Superoxide dismutase 1 (SOD1) was the first identified ALS gene, upon which mouse models resembling ALS have been generated and extensively studied 5 . In the spinal cord transverse section (Fig.…”
Section: Xpct For the Investigation Of Neurogenerative Diseasesmentioning
confidence: 99%