From: Pathology of the neurovascular unit in leukodystrophies
Patient | Expression pattern of protein of interest | |||||
---|---|---|---|---|---|---|
Endothelium | Basement membrane | Pericyte | Astrocyte | |||
Claudin-5 | ZO-1 | Laminin | PDGFRβ | AQP4 | α-dystroglycan | |
AGS | Unchanged | Redistribution | Unchanged | Unchanged | Redistribution | Low expression in vessels with low UEA I expressio |
AxD adolescent-onset | Unchanged | Redistribution | Unchanged | Unchanged | Redistribution | Low expression in vessels with low UEA I expression |
AxD infantile-onset | Unchanged | Redistribution | Unchanged | Unchanged | Redistribution | Low expression in vessels with low UEA I expression |
CARASAL | Unchanged | Redistribution | Unchanged | Unchanged | Redistribution | Unchanged |
ALSP | Unchanged | Unchanged | Unchanged | Unchanged | Redistribution | Low expression in vessels with low UEA I expression |
LARS2-related leukodystrophy | Unchanged | Redistribution | Expression in perivascular astrocytes | Unchanged | Redistribution | Unchanged |
MLD | Unchanged | Unchanged | Unchanged | Unchanged | Redistribution | Unchanged |
PMD | Unchanged | Unchanged | Unchanged | Unchanged | Redistribution | Unchanged |
X-ALD | Unchanged | Redistribution | Unchanged | Unchanged | Redistribution | Low expression in vessels with low UEA I expression |