Dysferlin is a new marker for leaky brain blood vessels in multiple sclerosis
Publisher DOI
PubMed ID
16957579
Abstract
Dysferlin is a muscle protein involved in cell membrane repair and its deficiency is associated with muscular dystrophy. We describe that dysferlin is also expressed in leaky endothelial cells. In the normal central nervous system (CNS), dysferlin is only present in endothelial cells of circumventricular organs. In the inflamed CNS of patients with multiple sclerosis (MS) or in animals with experimental autoimmune encephalomyelitis, dysferlin reactivity is induced in endothelial cells and the expression is associated with vascular leakage of serum proteins. In MS, dysferlin expression in endothelial cells is not restricted to vessels with inflammatory cuffs but is also present in noninflamed vessels. In addition, many blood vessels with perivascular inflammatory infiltrates lack dysferlin expression in inactive lesions or in the normal-appearing white matter. In vitro, dysferlin can be induced in endothelial cells by stimulation with tumor necrosis factor-alpha. Hence, dysferlin is not only a marker for leaky brain vessels, but also reveals dissociation of perivascular inflammatory infiltrates and blood-brain barrier disturbance in multiple sclerosis.
Date Issued
2006
Publication Type
Article
Language(s)
en
Author(s)
Hochmeister, Sonja | |
Grundtner, Roland | |
Bauer, Jan | |
Gordon, Grace | |
Korosec, Thomas | |
Kutzelnigg, Alexandra | |
Berger, Johannes J | |
Bradl, Monika | |
Bittner, Reginald E | |
Lassmann, Hans |
Additional Credits
Journal
Journal of neuropathology and experimental neurology
Publisher
Lippincott Williams & Wilkins
ISSN
0022-3069
ISBN
16957579
Access(Rights)
metadata.only