Merck
  • Home
  • Search Results
  • Is the SHRSP [corrected] strain a suitable model of spontaneous CADASIL?

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

Journal of molecular neuroscience : MN (2011-08-03)
Silvana Penco, Paolo Gelosa, Silvana Pileggi, Mauro Abbate, Alessandro Marocchi, Uliano Guerrini, Alice Pignieri, Elena Tremoli, Luigi Sironi
ABSTRACT

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.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Osmium tetroxide solution, suitable for electron microscopy, 2% in H2O
Sigma-Aldrich
Osmium tetroxide solution, suitable for electron microscopy, 4% in H2O
Sigma-Aldrich
Osmium tetroxide solution, 4 wt. % in H2O
Sigma-Aldrich
Osmium tetroxide solution, 2.5 wt. % in tert-butanol
Sigma-Aldrich
Osmium tetroxide, Sealed ampule.
Sigma-Aldrich
Os EnCat® 40, extent of labeling: 0.3 mmol/g Os loading
Sigma-Aldrich
Osmium tetroxide, ReagentPlus®, 99.8%
Sigma-Aldrich
Osmium tetroxide, ACS reagent, ≥98.0%