Table 1. Evidence of CNS barrier modifications in ALS from human biospecimens.
Parameter Observation References
Ultrastructure morphological changes in endothelium, astrocytes, pericytes Miyazaki 2011, Garbuzova-Davis 2012, Winkler 2013, Sasaki 2015, Yamadera 2015
↑ collagen content Garbuzova-Davis 2012, Sasaki 2015, Waters 2021
↓ collagen content Ono 1998, Miyazaki 2011
Tight junctions
well-preserved structure (electron microscopy)
Sasaki 2015
↓ mRNA or protein expression Henkel 2009, Garbuzova-Davis 2012
Leakiness blood derived protein detected in the spinal cord/cortex/CSF Donnefeld 1984, Leonardi 1984, Engelhardt 1990, Garbuzova-Davis 2012, Winkler 2013, Waters 2012
Efflux transporters P-gp
↑ (spinal cord homogenates)
Jablonski 2012
no change (spinal cord and motor cortex endothelium) ↑ (spinal cord and motor cortex astrocytes)
van Vliet 2019
BCRP ↑ in spinal cord homogenates Jablonski 2012
↑ (motor cortex endothelium); ↑ (spinal cord and motor cortex astrocytes)
van Vliet 2019