Details

Autor(en) / Beteiligte

• Penco, Silvana
• Gelosa, Paolo
• Pileggi, Silvana
• Abbate, Mauro
• Marocchi, Alessandro
• Guerrini, Uliano
• Pignieri, Alice
• Tremoli, Elena
• Sironi, Luigi

Titel

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

Ist Teil von

• Journal of molecular neuroscience, 2012-02, Vol.46 (2), p.427

Ort / Verlag

United States

Erscheinungsjahr

2012

Quelle

SpringerLink

Beschreibungen/Notizen

• 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.