Recently, a rare form of congenital muscular dystrophy has been shown to be associated with a deficiency of laminin a2 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 a2 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 a2 may be important for the selective filtration capability of the blood-brain barrier. The lack of laminin a2 in cerebral vessels of children with laminin a2-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 a2 deficiency to test this hypothesis.

Villanova, M., Malandrini, A., Sabatelli, P., Sewry, C.a., Toti, P., Torelli, S., et al. (1997). Localization of laminin alpha 2 chain in normal human central nervous system: an immunofluorescence and ultrastructural study. ACTA NEUROPATHOLOGICA, 94, 567-571.

Localization of laminin alpha 2 chain in normal human central nervous system: an immunofluorescence and ultrastructural study.

MALANDRINI, ALESSANDRO;TOTI, PAOLO;
1997

Abstract

Recently, a rare form of congenital muscular dystrophy has been shown to be associated with a deficiency of laminin a2 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 a2 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 a2 may be important for the selective filtration capability of the blood-brain barrier. The lack of laminin a2 in cerebral vessels of children with laminin a2-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 a2 deficiency to test this hypothesis.
Villanova, M., Malandrini, A., Sabatelli, P., Sewry, C.a., Toti, P., Torelli, S., et al. (1997). Localization of laminin alpha 2 chain in normal human central nervous system: an immunofluorescence and ultrastructural study. ACTA NEUROPATHOLOGICA, 94, 567-571.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11365/40662
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