Table 2.

Changes in blood–brain barrier and neurovascular unit component 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