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 (PMP22tg) Schwann cells in culture. In basal conditions, PMP22tg 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 PMP22tg Schwann to the axon or to axonal-mimicking stimuli significantly affects the transition of transgenic Schwann cells to the myelinating phenotype.
| Original language | English |
|---|---|
| Pages (from-to) | 263-273 |
| Number of pages | 11 |
| Journal | Neurobiology of Disease |
| Volume | 16 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Jun 2004 |
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Keywords
- Axon
- CMT1A
- Dysmyelination
- Hereditary neuropathy
- Migration
- Motility
- Myelin
- PMP22
- Proliferation
- Schwann cell
ASJC Scopus subject areas
- Neurology
Cite this
Impairment of PMP22 transgenic Schwann cells differentiation in culture : Implications for Charcot-Marie-Tooth type 1A disease. / Nobbio, Lucilla; Vigo, Tiziana; Abbruzzese, Michele; Levi, Giovanni; Brancolini, Claudio; Mantero, Stefano; Grandis, Marina; Benedetti, Luana; Mancardi, Gianluigi; Schenone, Angelo.
In: Neurobiology of Disease, Vol. 16, No. 1, 06.2004, p. 263-273.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Impairment of PMP22 transgenic Schwann cells differentiation in culture
T2 - Implications for Charcot-Marie-Tooth type 1A disease
AU - Nobbio, Lucilla
AU - Vigo, Tiziana
AU - Abbruzzese, Michele
AU - Levi, Giovanni
AU - Brancolini, Claudio
AU - Mantero, Stefano
AU - Grandis, Marina
AU - Benedetti, Luana
AU - Mancardi, Gianluigi
AU - Schenone, Angelo
PY - 2004/6
Y1 - 2004/6
N2 - 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 (PMP22tg) Schwann cells in culture. In basal conditions, PMP22tg 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 PMP22tg Schwann to the axon or to axonal-mimicking stimuli significantly affects the transition of transgenic Schwann cells to the myelinating phenotype.
AB - 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 (PMP22tg) Schwann cells in culture. In basal conditions, PMP22tg 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 PMP22tg Schwann to the axon or to axonal-mimicking stimuli significantly affects the transition of transgenic Schwann cells to the myelinating phenotype.
KW - Axon
KW - CMT1A
KW - Dysmyelination
KW - Hereditary neuropathy
KW - Migration
KW - Motility
KW - Myelin
KW - PMP22
KW - Proliferation
KW - Schwann cell
UR - http://www.scopus.com/inward/record.url?scp=2342530472&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=2342530472&partnerID=8YFLogxK
U2 - 10.1016/j.nbd.2004.02.007
DO - 10.1016/j.nbd.2004.02.007
M3 - Article
C2 - 15207283
AN - SCOPUS:2342530472
VL - 16
SP - 263
EP - 273
JO - Neurobiology of Disease
JF - Neurobiology of Disease
SN - 0969-9961
IS - 1
ER -