Demyelinating polyneuropathy in goats lacking prion protein.
FASEB J
; 34(2): 2359-2375, 2020 02.
Article
en En
| MEDLINE
| ID: mdl-31907995
ABSTRACT
Studies in mice with ablation of Prnp, the gene that encodes the cellular prion protein (PrPC ), have led to the hypothesis that PrPC is important for peripheral nerve myelin maintenance. Here, we have used a nontransgenic animal model to put this idea to the test; namely, goats that, due to a naturally occurring nonsense mutation, lack PrPC . Teased nerve fiber preparation revealed a demyelinating pathology in goats without PrPC . Affected nerves were invaded by macrophages and T cells and displayed vacuolated fibers, shrunken axons, and onion bulbs. Peripheral nerve lipid composition was similar in young goats with or without PrPC , but markedly different between corresponding groups of adult goats, reflecting the progressive nature of the neuropathy. This is the first report of a subclinical demyelinating polyneuropathy caused by loss of PrPC function in a nontransgenic mammal.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Polineuropatías
/
Cabras
/
Enfermedades Desmielinizantes
/
Proteínas PrPC
/
Vaina de Mielina
Límite:
Animals
Idioma:
En
Revista:
FASEB J
Asunto de la revista:
BIOLOGIA
/
FISIOLOGIA
Año:
2020
Tipo del documento:
Article
País de afiliación:
Noruega