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New insights into the molecular basis of progressive myoclonus epilepsy: a multiprotein complex with cystatin B (Articolo in rivista)
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- New insights into the molecular basis of progressive myoclonus epilepsy: a multiprotein complex with cystatin B (Articolo in rivista) (literal)
- Anno
- 2002-01-01T00:00:00+01:00 (literal)
- Alternative label
Di Giaimo R.1, Riccio M.2, Santi S.2, Galeotti C.3, Ambrosetti D.C.1, Melli M.1 (2002)
New insights into the molecular basis of progressive myoclonus epilepsy: a multiprotein complex with cystatin B
in Human molecular genetics (Print)
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- Di Giaimo R.1, Riccio M.2, Santi S.2, Galeotti C.3, Ambrosetti D.C.1, Melli M.1 (literal)
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- Lavoro multidisciplinare con implicazioni cliniche
I.F. 9,318 (literal)
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- Cystatin B is an anti-proteolytic polypeptide implicated in progressive myoclonus epilepsy (EPM1), a degenerative disease of the central nervous system. The knock-out mouse model of the disease shows apoptosis of the cerebellar granule cells. We have identified five recombinant proteins interacting with cystatin B and none of them is a protease. We show that three of these proteins (RACK-1, beta-spectrin and NF-L) co- immunoprecipitate with cystatin B in rat cerebellum. Confocal immunofluorescence analysis shows that the same proteins are present in the granule cells of developing cerebellum, as well as in Purkinje cells of adult rat cerebellum. We propose that a cystatin B multiprotein complex has a specific cerebellar function and that the loss of this function might contribute to the disease in EPM1 patients. (literal)
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- 1Dipartimento di Biologia - Uni. Bologna
2Istituto di Citomorfologia Normale e Patologica - CNR
3Chiron vaccines - Siena (literal)
- Titolo
- New insights into the molecular basis of progressive myoclonus epilepsy: a multiprotein complex with cystatin B (literal)
- Abstract
- Cystatin B is an anti-proteolytic polypeptide implicated in progressive
myoclonus epilepsy (EPM1), a degenerative disease of the central nervous
system. The knock-out mouse model of the disease shows apoptosis of the
cerebellar granule cells. We have identified five recombinant proteins
interacting with cystatin B and none of them is a protease. We show that
three of these proteins (RACK-1, beta-spectrin and NF-L) co-
immunoprecipitate with cystatin B in rat cerebellum. Confocal
immunofluorescence analysis shows that the same proteins are present in
the granule cells of developing cerebellum, as well as in Purkinje cells
of adult rat cerebellum. We propose that a cystatin B multiprotein complex
has a specific cerebellar function and that the loss of this function
might contribute to the disease in EPM1 patients. (literal)
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