Introduction: Cobalamin (Cbl) deficiency affects the peripheral nervous system (PNS) morphologically and functionally. We investigated whether the octapeptide repeat (OR)-region of prion protein (PrPC) (which is claimed to have myelinotrophic properties) is involved in the pathogenesis of rat Cbl-deficient (Cbl-D) polyneuropathy. Methods: We intracerebroventricularly administered antibodies (Abs) against the OR-region (OR-Abs) to Cbl-D rats to prevent myelin damage and maximum nerve conduction velocity (MNCV) abnormalities, and PrPCs to normal rats to reproduce PNS Cbl-D-like lesions. We measured nerve PrPC levels and MNCV. Results: The OR-Abs normalized myelin ultrastructure, MNCV values, and tumor necrosis factor(TNF)-α levels in the sciatic and tibial nerves of Cbl-D rats. PrPC levels increased in Cbl-D nerves. The nerves of the PrPC-treated rats showed typical Cbl-D lesions, significantly decreased MNCV values, and significantly increased TNF-α levels. Discussion: OR-Abs prevent the myelin damage caused by increased OR-regions, and excess TNF-α is involved in the pathogenesis of Cbl-D polyneuropathy.
The octapeptide repeat PrPC region and cobalamin-deficient polyneuropathy of the rat / G. Scalabrino, E. Mutti, D. Veber, V. Rodriguez Menendez, C. Novembrino, A. Calligaro, G. Tredici. - In: MUSCLE & NERVE. - ISSN 0148-639X. - 44:6(2011 Dec), pp. 957-967.
|Titolo:||The octapeptide repeat PrPC region and cobalamin-deficient polyneuropathy of the rat|
SCALABRINO, GIUSEPPE (Primo)
MUTTI, ELENA (Secondo)
|Parole Chiave:||Cellular prion protein; Cobalamin deficiency; Nerve conduction velocity; Peripheral nervous system; PrP c octapeptide repeat region|
|Settore Scientifico Disciplinare:||Settore MED/04 - Patologia Generale|
|Data di pubblicazione:||dic-2011|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1002/mus.22225|
|Appare nelle tipologie:||01 - Articolo su periodico|