Data di Pubblicazione:
2012
Citazione:
Is the SHRPS Strain a Suitable Model of Spontaneous CADASIL? / S. Penco, P. Gelosa, S. Pileggi, M. Abbate, A. Marocchi, U. Guerrini, A. Pignieri, E. Tremoli, L. Sironi. - In: JOURNAL OF MOLECULAR NEUROSCIENCE. - ISSN 0895-8696. - 46:2(2012 Feb), pp. 427-430. [10.1007/s12031-011-9605-4]
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.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
CADASIL; SHRSP; NOTCH3; Granular osmiophilic material; Animal model;
Elenco autori:
S. Penco, P. Gelosa, S. Pileggi, M. Abbate, A. Marocchi, U. Guerrini, A. Pignieri, E. Tremoli, L. Sironi
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