PMID: 9444358Jan 28, 1998Paper

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

Acta Neuropathologica
M VillanovaG C Guazzi

Abstract

Recently, a rare form of congenital muscular dystrophy has been shown to be associated with a deficiency of laminin alpha 2 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 alpha 2 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 alpha 2 may be important for the selective filtration capability of the blood-brain barrier. The lack of laminin alpha 2 in cerebral vessels of children with laminin alpha 2-deficient congenital muscular dystrophy may cause impaired selective filtration, leading to leakage of plasma components and damage to the CNS. Further studies sh...Continue Reading

Citations

Mar 8, 2005·Pediatric Radiology·Claudia C LeiteUmbertina C Reed
Jan 30, 2002·Pediatric Neurology·H Jacobus GilhuisFons J M Gabreëls
May 11, 2000·Pediatrics International : Official Journal of the Japan Pediatric Society·M TakahashiT Toyoda
Jul 5, 2012·Journal of Neuroinflammation·Kyungmin Ji, Stella E Tsirka
Sep 14, 2007·Acta Oto-laryngologica·Veronika StarlingerBernhard Schick
Mar 9, 2010·Neuromuscular Disorders : NMD·Fatemeh GeranmayehFrancesco Muntoni
Sep 8, 2004·Neuromuscular Disorders : NMD·Francesco Muntoni, Thomas Voit
Mar 31, 2009·Arquivos de neuro-psiquiatria·Umbertina Conti Reed
Jun 24, 2009·Arquivos de neuro-psiquiatria·Umbertina Conti Reed
Aug 28, 2019·The Journal of Comparative Neurology·Geoffroy NoëlHakima Moukhles
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Jun 9, 2020·Human Genome Variation·Keiko Yamamoto-ShimojimaToshiyuki Yamamoto
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Jan 10, 2021·Human Genome Variation·Keiko Yamamoto-ShimojimaToshiyuki Yamamoto
Jul 27, 2005·Trends in Neurosciences·Holly ColognatoMaria Laura Feltri

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