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Table 2 Changes in blood–brain barrier and neurovascular unit component in leukodystrophies

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