http://www.cnr.it/ontology/cnr/individuo/prodotto/ID49916
The spectrum of Notch3 mutations in 28 Italian CADASIL families (Articolo in rivista)
- Type
- Label
- The spectrum of Notch3 mutations in 28 Italian CADASIL families (Articolo in rivista) (literal)
- Anno
- 2005-01-01T00:00:00+01:00 (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
- 10.1136/jnnp.2004.048207 (literal)
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- M T Dotti1; A Federico1; R Mazzei2; S Bianchi1; O Scali1; F L Conforti2; T Sprovieri2; D Guidetti3; U Aguglia4; D Consoli5; L Pantoni6; C Sarti6; D Inzitari6; A Quattrone2 (literal)
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- 1 Department of Neurological and Behavioural Sciences, University of Siena, Siena, Italy
2 Institute of Neurological Sciences, National Research Council, Piano Lago di Mangone, Cosenza, Italy
3 Unit of Neurology, Reggio Emilia, Italy
4 Unit of Neurology, Reggio Calabria, Italy
5 Unit of Neurology, Vibo Valentia, Italy
6 Department of Neurological and Psychiatric Sciences, University of Florence, Italy (literal)
- Titolo
- The spectrum of Notch3 mutations in 28 Italian CADASIL families (literal)
- Abstract
- BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a cause of hereditary cerebrovascular disease. It results from mutations in the Notch3 gene, a large gene with 33 exons. A cluster of mutations around exons 3 and 4 was originally reported and limited scanning of these exons was suggested for the diagnosis in most cases. OBJECTIVE: To report Notch3 mutation analysis in 28 unrelated Italian CADASIL families from central and south Italy. RESULTS: The highest rate of mutations was found in exon 11 (21%) and only 18% of mutations were in exon 4. This may be related to the peculiar distribution of Notch3 mutations in the regions of origin of the families. CONCLUSIONS: The results suggest that limited scanning of exons 3 and 4 is inadvisable in CADASIL cases of Italian origin. (literal)
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