Impairment of PMP22 transgenig Schwann cells differentiatic in culture: implications for Charcot-Marie-Tooth type 1A disease (Articolo in rivista)

Type

• Prodotto della ricerca (Classe)
• Articolo in rivista (Classe)

Label

• Impairment of PMP22 transgenig Schwann cells differentiatic in culture: implications for Charcot-Marie-Tooth type 1A disease (Articolo in rivista) (literal)

Anno

• 2004-01-01T00:00:00+01:00 (literal)

Alternative label

• Nobbio l. 1,2, Vigo T. 1, Abbruzzese M. 1, Levi G. 3, Brancolini C. 4, Mantero S. 3,5, Grandis M. 1, Benedetti L. 1, Mancardi G 1,2., Schenone A.1,2 (2004)
  Impairment of PMP22 transgenig Schwann cells differentiatic in culture: implications for Charcot-Marie-Tooth type 1A disease
  in Neurobiology of disease
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Nobbio l. 1,2, Vigo T. 1, Abbruzzese M. 1, Levi G. 3, Brancolini C. 4, Mantero S. 3,5, Grandis M. 1, Benedetti L. 1, Mancardi G 1,2., Schenone A.1,2 (literal)

Pagina inizio

• 263 (literal)

Pagina fine

• 273 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 16 (literal)

Rivista

• Neurobiology of disease (Rivista)

Note

• ISI Web of Science (WOS) (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• 1. Department of Neurosciences, Ophthalmology and Genetics, University of Genova, Genova, Italy 2. Center of Excellence for Biomedical Research, University of Genova, Genova, Italy 3. Centre National de la Recherche Scientifique, Paris, France 4. Biology Section, Department of Biomedical Sciences and Technologies, University of Udine, Udine, Italy 5. Dulbecco Telethon Institute, CNR-ITB, Milan, Italy (literal)

Titolo

• Impairment of PMP22 transgenig Schwann cells differentiatic in culture: implications for Charcot-Marie-Tooth type 1A disease (literal)

Abstract

• Charcot-Marie-Tooth type 1A (CMT1A) is a hereditary demyelinating neuropathy due to an increased genetic dosage of the peripheral myelin protein 22 (PMP22). The mechanisms leading from PMP22 overexpression to impairment of myelination are still unclear. We evaluated expression and processing of PMP22, viability, proliferation, migration, motility and shaping properties, and ability of forming myelin of PMP22 transgenic (PMP22(tg)) Schwann cells in culture. In basal conditions, PMP22(tg) Schwann cells, although expressing higher PMP22 levels than control ones, show normal motility, migration and shaping properties. Addition of forskolin to the media induces an additional stimulation of PMP22 expression and results in an impairment of cells migration and motility, and a reduction of cell area and perimeter. Similarly, co-culturing transgenic Schwann cells with neurons causes an altered cells differentiation and an impairment of myelin formation. In conclusion, exposure of PMP22(tg) Schwann to the axon or to axonal-mimicking stimuli significantly affects the transition of transgenic Schwann cells to the myelinating phenotype. (literal)

Prodotto di

• Institute of molecular bioimaging and physiology (IBFM) (Istituto)

Autore CNR

• GIOVANNI LUIGI MANCARDI (Unità di personale esterno)
• MICHELE ABBRUZZESE (Unità di personale esterno)

Incoming links:

Prodotto

• Institute of molecular bioimaging and physiology (IBFM) (Istituto)

Autore CNR di

• GIOVANNI LUIGI MANCARDI (Unità di personale esterno)
• MICHELE ABBRUZZESE (Unità di personale esterno)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• Neurobiology of disease (Rivista)