Proteolipid protein is necessary in peripheral as well as central myelin.
Neuron
; 19(1): 205-18, 1997 Jul.
Article
em En
| MEDLINE
| ID: mdl-9247276
ABSTRACT
Alternative products of the proteolipid protein gene (PLP), proteolipid protein (PLP) and DM20, are major components of compact myelin in the central nervous system, but quantitatively minor constituents of Schwann cells. A family with a null allele of PLP has a less severe CNS phenotype than those with other types of PLP mutations. Moreover, individuals with PLP null mutations have a demyelinating peripheral neuropathy, not seen with other PLP mutations of humans or animals. Direct analysis of normal peripheral nerve demonstrates that PLP is localized to compact myelin. This and the clinical and pathologic observations of the PLP null phenotype indicate that PLP/DM20 is necessary for proper myelin function both in the central and peripheral nervous systems.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Sistema Nervoso Central
/
Córtex Cerebral
/
Doenças Desmielinizantes
/
Sistema Nervoso Periférico
/
Proteína Proteolipídica de Mielina
/
Proteínas da Mielina
Limite:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Humans
/
Middle aged
Idioma:
En
Revista:
Neuron
Assunto da revista:
NEUROLOGIA
Ano de publicação:
1997
Tipo de documento:
Article
País de afiliação:
Estados Unidos