ISSN:
1432-0533
Keywords:
Key words Basal lamina
;
Blood-brain barrier
;
Central nervous system
;
Congenital muscular dystrophy
;
Laminin α2
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Recently, a rare form of congenital muscular dystrophy has been shown to be associated with a deficiency of laminin α2 chain, a tissue-specific component of the basal lamina. Besides muscular dystrophy, children affected with this disorder also show electrophysiological and magnetic resonance imaging evidence of white matter involvement in the central nervous system (CNS). We have studied the precise localization of laminin α2 chain in normal human brain, using specific electron microscopic techniques including thin-section fracture labeling and cryoultramicrotomy, in parallel with immunohistochemical techniques. We found that this laminin chain was localized to the basal lamina of all cerebral blood vessels, whereas blood vessels of the choroid plexus did not show any reaction. No positive reaction was found in meningeal blood vessels either. We hypothesize that in normal brain, laminin α2 may be important for the selective filtration capability of the blood-brain barrier. The lack of laminin α2 in cerebral vessels of children with laminin α2-deficient congenital muscular dystrophy may cause impaired selective filtration, leading to leakage of plasma components and damage to the CNS. Further studies should be performed on patients affected by congenital muscular dystrophy associated with laminin α2 deficiency to test this hypothesis.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s004010050751
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