期刊
JOURNAL OF CELL BIOLOGY
卷 148, 期 5, 页码 1009-1020出版社
ROCKEFELLER UNIV PRESS
DOI: 10.1083/jcb.148.5.1009
关键词
PNS myelination; homophilic adhesion; dysmyelination; cell polarity; myelin protein gene dosage
类别
资金
- NINDS NIH HHS [NS-38186, NS-23375] Funding Source: Medline
This report investigated mechanisms responsible for failed Schwann cell myelination in mice that overexpress P-o (P-o(tg)), the major structural protein of PNS myelin. Quantitative ultrastructural immunocytochemistry established that P-o protein was mistargeted to abaxonal, periaxonal, and mesaxon membranes in P-o(tg) Schwann cells with arrested myelination. The extracellular leaflets of P-o-containing mesaxon membranes were closely apposed with periodicities of compact myelin. The myelin-associated glycoprotein was appropriately sorted in the Golgi apparatus and targeted to periaxonal membranes. In adult mice, occasional Schwann cells myelinated axons possibly with the aid of endocytic removal of mistargeted P-o. These results indicate that P-o gene multiplication causes P-o mistargeting to mesaxon membranes, and through obligate P-o homophilic adhesion, renders these dynamic membranes inert and halts myelination.
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