Skip to main content

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