Schwann cell expression of PLP1 but not DM20 is necessary to prevent neuropathy

Proteolipid protein (PLP1) and its alternatively spliced isoform, DM20, are the major myelin proteins in the CNS, but are also expressed in the PNS. The proteins have an identical sequence except for 35 amino acids in PLP1 (the PLP1‐specific domain) not present in DM20. Mutations of PLP1/DM20 cause...

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Veröffentlicht in:	Annals of neurology 2003-03, Vol.53 (3), p.354-365
Hauptverfasser:	Shy, Michael E., Hobson, Grace, Jain, Manisha, Boespflug-Tanguy, Odile, Garbern, James, Sperle, Karen, Li, Wen, Gow, Alex, Rodriguez, Diana, Bertini, Enrico, Mancias, Pedro, Krajewski, Karen, Lewis, Richard, Kamholz, John
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Amino Acid Sequence Animals Biological and medical sciences Cercopithecus aethiops Child Cohort Studies COS Cells Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Female Humans Male Medical sciences Molecular Sequence Data Mutation - genetics Myelin Proteolipid Protein - biosynthesis Myelin Proteolipid Protein - genetics Nerve Tissue Proteins Neurology Pelizaeus-Merzbacher Disease - genetics Pelizaeus-Merzbacher Disease - metabolism Pelizaeus-Merzbacher Disease - pathology Peripheral Nervous System Diseases - genetics Peripheral Nervous System Diseases - metabolism Peripheral Nervous System Diseases - pathology Rats Schwann Cells - metabolism
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