Is the SHRSP [corrected] strain a suitable model of spontaneous CADASIL?

A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral...

Full description

Saved in:
Bibliographic Details
Published in:Journal of molecular neuroscience 2012-02, Vol.46 (2), p.427
Main Authors: Penco, Silvana, Gelosa, Paolo, Pileggi, Silvana, Abbate, Mauro, Marocchi, Alessandro, Guerrini, Uliano, Pignieri, Alice, Tremoli, Elena, Sironi, Luigi
Format: Article
Language:English
Subjects:
Animals
Brain - pathology
CADASIL - genetics
Cytoplasmic Granules - chemistry
Cytoplasmic Granules - ultrastructure
Disease Models, Animal
Genetic Predisposition to Disease
Glomerular Basement Membrane - chemistry
Glomerular Basement Membrane - pathology
Humans
Hypertension - genetics
Hypertension - pathology
Magnetic Resonance Imaging
Male
Osmium Tetroxide
Proteinuria - genetics
Proteinuria - pathology
Rats
Rats, Inbred SHR - genetics
Rats, Mutant Strains - genetics
Rats, Sprague-Dawley
Receptor, Notch3
Receptors, Notch - analysis
Receptors, Notch - deficiency
Receptors, Notch - genetics
Sodium Chloride, Dietary - toxicity
Staining and Labeling
Stroke - etiology
Stroke - genetics
Stroke - pathology
Tunica Media - pathology
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A number of features of the pathology occurring in spontaneously hypertensive stroke prone rats (SHRSPs), such as MRI brain signal abnormalities, the presence of high protein content in cerebrospinal fluid and vessel wall thickening, seem to indicate that this strain is a suitable model for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). To explore this hypothesis, we sought the human diagnostic hallmarks of the disease [the accumulation of granular osmiophilic material (GOM) deposits in vessel walls and NOTCH3 gene mutations] in SHRSPs. Male SHRSPs fed a permissive diet were sacrificed 3 days after the first MRI visualisation of brain abnormalities. Whole blood and kidney samples were respectively collected for molecular and electron microscopy evaluations. Automated sequence analysis of exons and intron-exon boundaries did not reveal any genetic variation in the NOTCH3 gene, and electron microscopy excluded the presence of GOM. The findings of this study exclude SHRSPs as a possible model for CADASIL.
ISSN:1559-1166
DOI:10.1007/s12031-011-9605-4