Dysferlin is a new marker for leaky brain blood vessels in multiple sclerosis

Hochmeister, Sonja; Grundtner, Roland; Bauer, Jan; Engelhardt, Britta; Lyck, Ruth; Gordon, Grace; Korosec, Thomas; Kutzelnigg, Alexandra; Berger, Johannes J; Bradl, Monika; Bittner, Reginald E; Lassmann, Hans (2006). Dysferlin is a new marker for leaky brain blood vessels in multiple sclerosis. Journal of neuropathology and experimental neurology, 65(9), pp. 855-65. Hagerstown, Md.: Lippincott Williams & Wilkins 10.1097/01.jnen.0000235119.52311.16

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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.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Pre-clinic Human Medicine > Theodor Kocher Institute
UniBE Contributor:	Engelhardt, Britta, Lyck, Ruth
ISSN:	0022-3069
ISBN:	16957579
Publisher:	Lippincott Williams & Wilkins
Language:	English
Submitter:	Factscience Import
Date Deposited:	04 Oct 2013 14:45
Last Modified:	05 Dec 2022 14:14
Publisher DOI:	10.1097/01.jnen.0000235119.52311.16
PubMed ID:	16957579
Web of Science ID:	000241692400003
URI:	https://boris.unibe.ch/id/eprint/18593 (FactScience: 793)